S. Zierz

K. Koehler, A. Huebner, S. Zierz, 2008, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

Ecto- and cytosolic 5′-nucleotidases in normal and AMP deaminase-deficient human skeletal muscle

Y. Hellsten, F. Hanisch, S. Zierz, 2006, Biological chemistry.

C-Reactive Protein in Idiopathic Inflammatory Myopathies

F. Hanisch, S. Zierz, 2015 .

Endocrine abnormalities in mitochondrial myopathy with external ophthalmoplegia

D. Klingmüller, S. Zierz, A. Quader, 1992, The clinical investigator.

Partial deficiency of complexes I and IV of the mitochondrial respiratory chain in skeletal muscle of two patients with mitochondrial myopathy

S. Zierz, J. Bleistein, 1989, Journal of Neurology.

Update Distale Myopathien

T. Kraya, S. Zierz, D. Stoevesandt, 2022, Klinische Neurophysiologie.

The Effect of Resveratrol on Mitochondrial Function in Myoblasts of Patients with the Common m.3243A>G Mutation

F. Dehghani, Diana Lehmann Urban, Leila Motlagh Scholle, 2020, Biomolecules.

Higher proportion of mitochondrial A3243G mutation in blood than in skeletal muscle in a patient with cardiomyopathy and hearing loss.

S. Zierz, S. Neudecker, F. Gellerich, 2000, Molecular genetics and metabolism.

Understanding mitochondrial DNA maintenance disorders at the single muscle fibre level

Robert W. Taylor, D. Turnbull, C. Lawless, 2019, Nucleic acids research.

Treatment of adult idiopathic inflammatory myopathies with conventional immunosuppressive drugs

M. Kornhuber, S. Zierz, G. Keyßer, 2019, Zeitschrift für Rheumatologie.

Neuroleptic associated extrapyramidal symptoms.

S. Zierz, F. Jerusalem, I. Maurer, 1995, The British journal of psychiatry : the journal of mental science.

Impaired Calcium Homeostasis and Calcium Antagonists in Muscle Diseases

S. Zierz, 1989 .

Attitudes towards complementary and alternative medicine in chronic pain syndromes: a questionnaire-based comparison between primary headache and low back pain

C. Gaul, S. Zierz, T. Schmidt, 2011, BMC complementary and alternative medicine.

Use of Complementary and Alternative Medicine in Patients Suffering from Primary Headache Disorders

A. May, S. Evers, C. Gaul, 2009, Cephalalgia : an international journal of headache.

Are there two forms of carnitine palmitoyltransferase in muscle?

A. Engel, S. Zierz, 1987, Neurology.

Effects of extramitochondrial ADP on permeability transition of mouse liver mitochondria.

S. Zierz, F. Gellerich, Z. Gizatullina, 2005, Biochimica et biophysica acta.

Increased Blood Flow Velocity in Middle Cerebral Artery and Headache Upon Ingestion of Ice Water

A. Wienke, T. Kraya, S. Zierz, 2019, Front. Neurol..

Headache and migraine in mitochondrial disease and its impact on life—results from a cross-sectional, questionnaire-based study

T. Kraya, S. Watzke, S. Zierz, 2021, Acta Neurologica Belgica.

Prevalence and characteristics of headache attributed to ingestion or inhalation of a cold stimulus (HICS): A cross-sectional study

T. Kraya, S. Watzke, S. Zierz, 2020, Cephalalgia : an international journal of headache.

Myocardial respiratory chain enzyme activities in idiopathic dilated cardiomyopathy, and comparison with those in atherosclerotic coronary artery disease and valvular aortic stenosis.

S. Zierz, I. Maurer, 1993, The American journal of cardiology.

Profile of alglucosidase alfa in the treatment of Pompe disease: safety, efficacy, and patient acceptability

S. Zierz, I. Schneider, 2016 .

Respiratory function in late-onset Pompe disease patients receiving long-term enzyme replacement therapy for more than 48 months

F. Hanisch, S. Zierz, T. Müller, 2012, Wiener Medizinische Wochenschrift.

Lack of dystrophin is associated with altered integration of the mitochondria and ATPases in slow-twitch muscle cells of MDX mice.

L. Kadaja, M. Eimre, H. Jockusch, 2001, Biochimica et biophysica acta.

Coenzyme Q in serum and muscle of 5 patients with Kearns-Sayre syndrome and 12 patients with ophthalmoplegia plus

S. Zierz, F. Jerusalem, G. Jahns, 1989, Journal of Neurology.

Central and peripheral nervous system conduction in mitochondrial myopathy with chronic progressive external ophthalmoplegia.

R. Dengler, M. Schubert, S. Zierz, 1994, Electroencephalography and clinical neurophysiology.

Progressive Muscle Dystrophies

S. Zierz, 2010 .

Lactate increase and oxygen desaturation in mitochondrial disorders – Evaluation of two diagnostic screening protocols

F. Hanisch, S. Zierz, M. Deschauer, 2006, Journal of Neurology.

Impaired Regulation of Brain Mitochondria by Extramitochondrial Ca2+ in Transgenic Huntington Disease Rats*

B. Landwehrmeyer, O. Riess, F. Striggow, 2008, Journal of Biological Chemistry.

A novel nonsense mutation (R269X) in the myophosphorylase gene in a patient with McArdle disease.

A. Lindner, S. Zierz, M. Deschauer, 2001, Molecular genetics and metabolism.

Thymic pathologies in myasthenia gravis: a preoperative assessment of CAT scan and nuclear based imaging

S. Zierz, K. Jordan, M. Bähre, 2016, Journal of Neurology.

Molecular diagnosis of German patients with late-onset glycogen storage disease type II

M. Vorgerd, S. Zierz, K. Eger, 2008, Journal of Inherited Metabolic Disease.

“Thoracic migraine” as a new manifestation of migraine: Case report

T. Kraya, S. Zierz, S. Mages, 2018, Cephalalgia : an international journal of headache.

Frequency of Headache in Mitochondrial Disorders: A Response

T. Kraya, S. Zierz, 2018, Headache.

Prevalence of Headache in Patients With Mitochondrial Disease: A Cross‐Sectional Study

T. Kraya, C. Gaul, S. Zierz, 2018, Headache.

Different sites of inhibition of carnitine palmitoyltransferase by malonyl-CoA, and by acetyl-CoA and CoA, in human skeletal muscle.

A. Engel, S. Zierz, 1987, The Biochemical journal.

Foodborne botulism due to ingestion of home-canned green beans: two case reports

S. Zierz, K. Wartenberg, Tobias Mueller, 2018, Journal of Medical Case Reports.

A family with PROMM not linked to the recently mapped PROMM locus DM2

S. Zierz, K. Eger, T. Wieser, 2000, Neuromuscular Disorders.

Brainstem infarctions with normal MRI.

M. Krasnianski, A. Lindner, S. Zierz, 2002, European journal of medical research.

Hot-spot KIF5A mutations cause familial ALS

H. Braak, T. Meitinger, T. Strom, 2018, Brain : a journal of neurology.

Erenumab for migraine prevention in a patient with mitochondrial encephalopathy, lactate acidosis, and stroke‐like episodes syndrome: A case report

T. Kraya, S. Zierz, D. Holle, 2021, Headache.

Testicular dysfunction in adrenomyeloneuropathy.

D. Klingmüller, S. Zierz, W. Köhler, 1997, European journal of endocrinology.

Distal myopathies: from clinical classification to molecular understanding

T. Kraya, S. Zierz, Stephan Zierz, 2013, Journal of Neural Transmission.

Impaired energy metabolism in hearts of septic baboons: diminished activities of Complex I and Complex II of the mitochondrial respiratory chain.

K. Werdan, H. Redl, G. Schlag, 1999, Shock.

Mitochondrial encephalomyopathy: correlation of P-31 exercise MR spectroscopy with clinical findings.

C. Kuhl, M. Reiser, G. Layer, 1994, Radiology.

Cerebral MR in ophthalmoplegia plus.

M. Reiser, C. Leutner, G. Layer, 1994, AJNR. American journal of neuroradiology.

Relation between maximum discharge rates on electromyography and motor unit number estimates

S. Zierz, D. Georgiadis, W. Schulte-Mattler, 2000, Muscle & nerve.

[Congenital fibrosis of extraocular muscles (CFEOM) and other phenotypes of congenital cranial dysinnervation syndromes (CCDD)].

F. Hanisch, S. Zierz, V. Bau, 2005, Der Nervenarzt.

Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle.

A. Engel, S. Zierz, 1985, European journal of biochemistry.

Sialylation and Muscle Performance: Sialic Acid Is a Marker of Muscle Ageing

H. Holzhausen, J. Weis, F. Hanisch, 2013, PloS one.

CHLAMYDIA INFECTION

A. L. Wong, J. Belmont, F. Lammert, 2001 .

Characterization of Gait and Postural Regulation in Late-Onset Pompe Disease

K. Laudner, K. Delank, S. Schulze, 2020, Applied Sciences.

Images in clinical medicine. Retroperitoneal hemorrhage.

A. Lindner, S. Zierz, 2001, The New England journal of medicine.

Carnitine palmitoyltransferase II deficiency

K. Olek, S. Zierz, T. Wieser, 2003, Neurology.

Molecular and biochemical investigations in fumarase deficiency.

A. Schulze, S. Zierz, M. Pritsch, 2006, Molecular genetics and metabolism.

Muscle Carnitine Palmitoyltransferase II Deficiency: A Review of Enzymatic Controversy and Clinical Features

D. Robaa, S. Zierz, L. Motlagh, 2017, International journal of molecular sciences.

Detection of Mitochondrial Diseases

S. Zierz, F. Gellerich, Colloquium on Mitochondria, 1997, Developments in Molecular and Cellular Biochemistry.

Utility of a next-generation sequencing-based gene panel investigation in German patients with genetically unclassified limb-girdle muscular dystrophy

B. Schoser, S. Wenninger, S. Zierz, 2016, Journal of Neurology.

Frequency of calpain-3 c.550delA mutation in limb girdle muscular dystrophy type 2 and isolated hyperCKemia in German patients.

F. Hanisch, S. Zierz, K. Traufeller, 2007, Clinical neuropathology.

Behandelbarkeit der „sporadic late onset nemaline myopathy“

F. Hanisch, B. Romeike, S. Zierz, 2013, Der Nervenarzt.

Unusual manifestations in two cases of necrotizing myopathy associated with SRP-antibodies

F. Hanisch, S. Zierz, G. Stoltenburg, 2012, Clinical Neurology and Neurosurgery.

Unusual manifestations in two cases of necrotizing myopathy associated with SRP antibodies

F. Hanisch, S. Zierz, G. Stoltenburg, 2013, Rheumatology International.

Frequencies of myohistological mitochondrial changes in patients with mitochondrial DNA deletions and the common m.3243A>G point mutation

S. Zierz, P. Joshi, C. Zierz, 2015, Neuropathology : official journal of the Japanese Society of Neuropathology.

Myositis in Lewis rats induced by the superantigen Staphylococcal enterotoxin A

M. Kornhuber, K. Delank, S. Zierz, 2019, Molecular Biology Reports.

[Facioscapulohumeral muscular dystrophy. Clinical picture, atypical forms, diagnostics, genetics].

S. Zierz, C. Müller-Reible, B. Jordan, 2011, Der Nervenarzt.

Bi-allelic truncating mutations in VWA1 cause neuromyopathy.

B. Schlotter-Weigel, T. Meitinger, T. Strom, 2021, Brain : a journal of neurology.

The problem of interlab variation in methods for mitochondrial disease diagnosis: enzymatic measurement of respiratory chain complexes.

W. Sperl, S. Reuter, S. Zierz, 2004, Mitochondrion.

Muscle Carnitine Palmitoyltransferase II (CPT II) Deficiency: A Conceptual Approach

S. Zierz, P. Joshi, 2020, Molecules.

Compound heterozygosity for metachromatic leukodystrophy and arylsulfatase A pseudodeficiency alleles is not associated with progressive neurological disease.

H. Moser, J. Kappler, P. Propping, 1993, American journal of human genetics.

Normal activities of AMP-deaminase and adenylate kinase in patients with McArdle disease

S. Zierz, P. Joshi, T. Apitz, 2016, Neurological research.

AMP deaminase deficiency in skeletal muscle is unlikely to be of clinical relevance

F. Hanisch, S. Zierz, P. Joshi, 2006, Neuromuscular Disorders.

Normal Thermostability of p.Ser113Leu and p.Arg631Cys Variants of Mitochondrial Carnitine Palmitoyltransferase II (CPT II) in Human Muscle Homogenate

E. Pegoraro, S. Zierz, P. Joshi, 2022, Metabolites.

Lack of activation of the S113L variant of carnitine palmitoyltransfersase II by cardiolipin

Leila Motlagh Scholle, S. Zierz, M. Beckers, 2018, Journal of Bioenergetics and Biomembranes.

Biochemical evidence for heterozygosity in muscular carnitine palmitoyltransferase deficiency

R. R. Mundegar, S. Zierz, F. Jerusalem, 1993, The clinical investigator.

Bruns syndrome caused by intraventricular tumor.

M. Krasnianski, S. Zierz, T. Müller, 2007, European journal of medical research.

Inclusion body myositis : clinical and histopathological features of 36 patients

S. Beyenburg, S. Zierz, F. Jerusalem, 1993, The clinical investigator.

Normal protein content but abnormally inhibited enzyme activity in muscle carnitine palmitoyltransferase II deficiency

S. Zierz, D. Lehmann, 2014, Journal of the Neurological Sciences.

Predictive value of S-100beta and neuron-specific enolase serum levels for adverse neurologic outcome after cardiac surgery.

C. Lautenschläger, T. Deufel, A. Lindner, 2000, The Journal of thoracic and cardiovascular surgery.

Therapy of neurological disorders in systemic vasculitis.

R. Gold, S. Zierz, A. Fontana, 2003, Seminars in neurology.

Typical facioscapulohumeral dystrophy phenotype in patients without FSHD 4q35 deletion

M. Krasnianski, S. Zierz, K. Eger, 2003, Journal of Neurology.

High-altitude cerebral edema or acute demyelinating encephalomyelitis in the Himalayas.

T. Kraya, S. Zierz, P. Presek, 2018, Neurology. Clinical practice.

Exon skipping in Duchenne Muscle dystrophy due to a silent p.Ser443= mutation in the DMD gene

S. Zierz, D. Gläser, P. Joshi, 2020, Journal of Clinical Neuroscience.

Genetic heterogeneity in 30 German patients with oculopharyngeal muscular dystrophy

S. Zierz, M. Deschauer, M. Deschauer, 2006, Journal of Neurology.

GCG Repeats and phenotype in oculopharyngeal muscular dystrophy

S. Zierz, R. Schröder, T. Müller, 2001, Muscle & nerve.

Chronic Progressive External Ophthalmoplegia – A Common Ocular Manifestation of Mitochondrial Disorders

S. Zierz, M. Deschauer, 2008 .

A novel ANT1 gene mutation with probable germline mosaicism in autosomal dominant progressive external ophthalmoplegia

Robert W. Taylor, P. Chinnery, G. Hudson, 2005, Neuromuscular Disorders.

Energetic depression caused by mitochondrial dysfunction

S. Trumbeckaite, S. Zierz, F. Gellerich, 2004, Molecular and Cellular Biochemistry.

A novel Twinkle gene mutation in autosomal dominant progressive external ophthalmoplegia

Robert W. Taylor, D. Turnbull, R. Kiefer, 2003, Neuromuscular Disorders.

SANDO syndrome in a cohort of 107 patients with CPEO and mitochondrial DNA deletions

Robert W. Taylor, M. Kornhuber, F. Hanisch, 2014, Journal of Neurology, Neurosurgery & Psychiatry.

Sensory ataxic neuropathy due to a novel C10Orf2 mutation with probable germline mosaicism

P. Chinnery, G. Hudson, S. Zierz, 2005, Neurology.

Compartmentation of energy metabolism in atrial myocardium of patients undergoing cardiac surgery

M. Eimre, V. Saks, S. Zierz, 2005, Molecular and Cellular Biochemistry.

[Ophthalmoplegia-plus: clinical variability, biochemical defects of the mitochondria respiratory chain and deletions of the mitochondria genome].

K. Gerbitz, S. Zierz, F. Jerusalem, 1990, Der Nervenarzt.

Atypical phenotypes in patients with facioscapulohumeral muscular dystrophy 4q35 deletion.

M. Krasnianski, S. Zierz, K. Eger, 2003, Archives of neurology.

Myocardial respiratory chain enzyme activities in anomalous origin of the left main coronary artery from the pulmonary trunk (Bland-White-Garland syndrome) and comparison with atherosclerotic coronary artery disease.

S. Zierz, I. Maurer, 1992, The American journal of cardiology.

Successful treatment of MuSK antibody–positive myasthenia gravis with rituximab

S. Zierz, K. Jordan, M. Deschauer, 2006, Muscle & nerve.

TIA1 variant drives myodegeneration in multisystem proteinopathy with SQSTM1 mutations

J. Taylor, H. Goebel, P. Chinnery, 2018, The Journal of clinical investigation.

Heteroplasmy and Copy Number in the Common m.3243A>G Mutation—A Post-Mortem Genotype–Phenotype Analysis

C. Mawrin, Diana Lehmann Urban, Leila Motlagh Scholle, 2020, Genes.

A novel m.7539C>T point mutation in the mt-tRNAAsp gene associated with multisystemic mitochondrial disease

Robert W. Taylor, S. Zierz, P. Joshi, 2015, Neuromuscular Disorders.

A specific point mutation in the mitochondrial genome of Caucasians with MELAS

K. Gerbitz, D. Pongratz, S. Zierz, 1991, Human Genetics.

Respiratory chain activity in tissues from patients (MELAS) with a point mutation of the mitochondrial genome [tRNALeu(UUR)]

K. Gerbitz, S. Zierz, J. Müller‐Höcker, 1991, FEBS letters.

Adolph Seeligmüller's contribution to myotonia congenita Thomsen

S. Zierz, Carolin Arendt, 2020, Neuromuscular Disorders.

Mutations in the gene for X-linked adrenoleukodystrophy in patients with different clinical phenotypes.

A. Braun, J. Gärtner, A. Roscher, 1995, American journal of human genetics.

External ophthalmoplegia due to ocular myositis in a patient with ophthalmic herpes zoster

M. Krasnianski, S. Zierz, M. Sievert, 2004, Neuromuscular Disorders.

Cauda equina syndrome with multiple lumbar diverticula complicating long-standing ankylosing spondylitis

H. Urbach, S. Zierz, R. Schröder, 1994, The clinical investigator.

The quantitation of ADP diffusion gradients across the outer membrane of heart mitochondria in the presence of macromolecules.

K. Nicolay, S. Zierz, F. Gellerich, 2002, Biochimica et biophysica acta.

Physical fatigability and muscle pain in patients with Hashimoto thyroiditis

S. Zierz, B. Jordan, A. Spens, 2021, Journal of Neurology.

Different sensitivities of CPT I and CPT II for inhibition by l-aminocarnitine in human skeletal muscle.

S. Zierz, K. Traufeller, F. Gellerich, 2004, Biochimica et biophysica acta.

Genetics of carnitine palmitoyltransferase II deficiencies.

S. Zierz, T. Wieser, M. Deschauer, 1999, Advances in experimental medicine and biology.

Kinetic investigation of carnitine palmitoyltransferases in homogenates of human skeletal muscle using L-amino-carnitine and malonyl-CoA.

S. Zierz, F. Gellerich, W. Hein, 1999, Advances in experimental medicine and biology.

The forgotten condyle: Delayed hypoglossal nerve palsy caused by fracture of the occipital condyle

A. Lindner, S. Zierz, S. Demisch, 1998, Clinical Neurology and Neurosurgery.

A Randomized, Double Blind, Placebo-Controlled Trial of Pioglitazone in Combination with Riluzole in Amyotrophic Lateral Sclerosis

U. Bogdahn, C. Wessig, A. Winkler, 2012, PloS one.

Stabilization of the thermolabile variant S113L of carnitine palmitoyltransferase II

W. Sippl, R. Golbik, S. Zierz, 2016, Neurology: Genetics.

Malony-CoA inhibits the S113L variant of carnitine-palmitoyltransferase II.

W. Sippl, R. Golbik, S. Zierz, 2016, Biochimica et biophysica acta.

Ice cream headache in students and family history of headache: a cross-sectional epidemiological study

A. Wienke, T. Kraya, S. Zierz, 2016, Journal of Neurology.

Influence of transducer frequency on Doppler microemboli signals in an in vivo model.

A. Lindner, S. Zierz, D. Georgiadis, 1998, Neurological research.

Automated intraoperative detection of Doppler microembolic signals using the bigate approach.

A. Lindner, S. Zierz, D Georgiadis, 1998, Stroke.

Venous Microemboli in Patients with Artificial Heart Valves

R. Baumgartner, A. Lindner, S. Zierz, 1999, Cerebrovascular Diseases.

Time course of high-intensity transient signals in patients undergoing elective heart valve replacement: a prospective study.

A. Lühmann, A. Lindner, S. Zierz, 1997, The Journal of heart valve disease.

Different K+-release in distal myogenic and neurogenic muscular weakness during non-ischemic exercise

F. Markwardt, S. Zierz, S. Naegel, 2021, Journal of the Neurological Sciences.

The concept and diagnostic criteria of primary lateral sclerosis

G. Nagel, S. Petri, J. Kassubek, 2017, Acta neurologica Scandinavica.

C19orf12 mutations in neurodegeneration with brain iron accumulation mimicking juvenile amyotrophic lateral sclerosis

H. Prokisch, T. Haack, C. Gaul, 2012, Journal of Neurology.

Activation of epileptic foci by transcranial magnetic stimulation: effects on secretion of prolactin and luteinizing hormone

C. Elger, A. Hufnagel, D. Klingmüller, 1990, Journal of Neurology.

Evidence for a mitochondrial oxidative phosphorylation defect in brains from patients with schizophrenia

H. Möller, S. Zierz, I. Maurer, 2001, Schizophrenia Research.

Cognitive impairment, clinical severity and MRI changes in MELAS syndrome.

T. Kraya, S. Watzke, Y. Paelecke-Habermann, 2017, Mitochondrion.

A selective defect of cytochrome c oxidase is present in brain of Alzheimer disease patients

H. Möller, S. Zierz, I. Maurer, 2000, Neurobiology of Aging.

Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.

J. Dreyhaupt, J. Kassubek, A. Ludolph, 2016, Brain : a journal of neurology.

Abnormal dystrophin expression in patients with limb girdle syndromes

S. Beyenburg, R. R. Mundegar, S. Zierz, 1994, Journal of Neurology.

Frequency of the FKRP mutation c.826C>A in isolated hyperCKemia and in limb girdle muscular dystrophy type 2 in German patients

F. Hanisch, S. Zierz, M. Deschauer, 2010, Journal of Neurology.

[The clinical spectrum of limb-girdle muscular dystrophies type 2I in cases of a mutation in the "fukutin-related- protein"-gene].

M. Krasnianski, S. Zierz, S. Neudecker, 2004, Der Nervenarzt.

Increased laminin a expression in regenerating myofibers neuromuscular disorders

R. R. Mundegar, S. Zierz, J. von Oertzen, 1995, Muscle & nerve.

Diagnostic impact of myotonic discharges in myofibrillar myopathies

M. Kornhuber, T. Kraya, F. Hanisch, 2013, Muscle & nerve.

Phenotype of matrin‐3–related distal myopathy in 16 German patients

M. Kornhuber, J. Weis, T. Kraya, 2014, Annals of neurology.

The significance of pathological spontaneous activity in various myopathies

M. Kornhuber, F. Hanisch, S. Zierz, 2014, Clinical Neurophysiology.

Mutant carnitine palmitoyltransferase associated with myoadenylate deaminase deficiency in skeletal muscle.

S. Zierz, C. Reuschenbach, Christine Reuschenbach, 1988, The Journal of pediatrics.

Report of a Familial Incidence

S. Zierz, I. Maurer, 2016 .

Effect of Recanalization on Cerebral Edema, Long-Term Outcome, and Quality of Life in Patients with Large Hemispheric Infarctions.

S. Zierz, Silvio Brandt, K. Wartenberg, 2020, Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.

Acute Cerebrovascular Disease in the Young: The Stroke in Young Fabry Patients Study

G. Deuschl, A. Metz, D. Krieger, 2013, Stroke.

Myotonic dystrophy and limb girdle muscular dystrophy in one family

S. Beyenburg, K. Olek, S. Zierz, 1994, The clinical investigator.

Positive correlation between aortic valve pressure gradient and mitochondrial respiratory chain capacity in hypertrophied human left ventricle

S. Zierz, I. Maurer, 1992, The clinical investigator.

Mycophenolate mofetil as second line immunosuppressant in myasthenia gravis - a long-term prospective open-label study

F. Hanisch, S. Zierz, M. Wendt, 2009, European Journal of Medical Research.

Histological characterization and biochemical analysis of paraspinal muscles in neuromuscularly healthy subjects

J. Kassubek, A. Ludolph, A. Rosenbohm, 2015, Muscle & nerve.

TIA 1 variant drives myodegeneration in multisystem proteinopathy with SQSTM 1 mutations

J. Taylor, H. Goebel, P. Chinnery, 2018 .

MELAS ASSOCIATED WITH MUTATIONS IN THE POLG1 GENE

D. Turnbull, T. Kraya, S. Zierz, 2007, Neurology.

MELAS/SANDO overlap syndrome associated with POLG1 mutations

I. Wanke, Niels Hansen, S. Zierz, 2012, Neurological Sciences.

Mitochondrial 3243 A→G mutation (MELAS mutation) associated with painful muscle stiffness

A. Lindner, S. Zierz, S. Neudecker, 1999, Neuromuscular Disorders.

Pathogenic mitochondrial mt-tRNAAla variants are uniquely associated with isolated myopathy

Robert W. Taylor, S. Zierz, P. Joshi, 2015, European Journal of Human Genetics.

Coenzyme Q10 and respiratory chain enzyme activities in hypertrophied human left ventricles with aortic valve stenosis.

S. Zierz, A. Bernhard, I. Maurer, 1990, American Journal of Cardiology.

FGF-21 as a Potential Biomarker for Mitochondrial Diseases.

T. Kraya, S. Zierz, M. Deschauer, 2018, Current medicinal chemistry.

Mitochondrial respiratory chain enzyme activities in tetralogy of Fallot

S. Zierz, I. Maurer, 1994, The clinical investigator.

Late-onset encephalopathy associated with a C11777A mutation of mitochondrial DNA

D. Claus, S. Zierz, M. Deschauer, 2003, Neurology.

Carnitine acyltransferases in normal human skeletal muscle and in muscle of patients with carnitine palmitoyltransferase deficiency

S. Zierz, S. Neumann-Schmidt, 1991, Neuromuscular Disorders.

Startle-induced seizures and their relationship to epilepsy: Three case reports

C. Elger, S. Zierz, H. Penin, 1990 .

Thrombocytopenia induced by erucic acid therapy in patients with X-linked adrenoleukodystrophy

S. Zierz, C. Unkrig, R. Schröder, 1993, Clinical Investigation.

A novel nonsense mutation (515del4) in muscle carnitine palmitoyltransferase II deficiency.

S. Zierz, R. Schröder, T. Wieser, 2002, Molecular genetics and metabolism.

Carnitine palmitoyltransferase II (CPT II) deficiency: Genotype–Phenotype analysis of 50 patients

S. Zierz, P. Joshi, M. Deschauer, 2014, Journal of the Neurological Sciences.

Expanding mutation spectrum in CPT II gene: identification of four novel mutations

S. Zierz, P. Joshi, M. Deschauer, 2013, Journal of Neurology.

Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects.

S. Zierz, T. Wieser, M. Deschauer, 2005, Archives of neurology.

A splice junction mutation in muscle carnitine palmitoyltransferase II deficiency.

Robert W. Taylor, D. Turnbull, Z. Chrzanowska-Lightowlers, 2003, Molecular genetics and metabolism.

New Insights into the Metabolic Consequences of Large‐Scale mtDNA Deletions: A Quantitative Analysis of Biochemical, Morphological, and Genetic Findings in Human Skeletal Muscle

C. Elger, H. Reichmann, T. Klockgether, 2000, Journal of neuropathology and experimental neurology.

Differentiation between true microembolic signals and artefacts using an arbitrary sample volume.

A. Lindner, S. Zierz, D Georgiadis, 2000, Ultrasound in medicine & biology.

Effect of the new matrix metalloproteinase inhibitor RO-28-2653 on mitochondrial function.

Stephan Zierz, S. Zierz, F. Gellerich, 2002, Biochemical pharmacology.

Increased metabolic muscle fatigue is caused by some but not all mitochondrial mutations.

P. Iaizzo, S. Zierz, F. Gellerich, 2003, Archives of neurology.

Muscle fatigue, lactate, and pyruvate in mitochondrial myopathy with progressive external ophthalmoplegia

K. Wohlfarth, R. Dengler, M. Schubert, 1996, Muscle & nerve.

Ultrastructural abnormalities of mitochondria and deficiency of myocardial cytochromec oxidase in a patient with ventricular tachycardia

B. Strauer, M. Block, K. Reiners, 2005, Virchows Archiv A.

Mitochondrial myopathies: divergences of genetic deletions, biochemical defects and the clinical syndromes

K. Gerbitz, D. Pongratz, P. Lestienne, 2004, Journal of Neurology.

Low stability of huntington muscle Mitochondria against Ca2+ in R6/2 mice

B. Landwehrmeyer, K. Lindenberg, A. Ludolph, 2006, Annals of neurology.

Treatment of tension-type headache with botulinum toxin: a pilot study.

S. Zierz, T. Wieser, W. Schulte-Mattler, 1999, European journal of medical research.

Acute compartment syndrome after forearm ischemic work test in a patient with McArdle’s disease

A. Lindner, S. Zierz, N. Reichert, 2001, Neurology.

Analysis of spectrum and frequencies of mutations in McArdle disease

M. Vorgerd, P. Chinnery, H. Schreiber, 2007, Journal of Neurology.

McArdle Disease: Clinical, Biochemical, Histological and Molecular Genetic Analysis of 60 Patients

S. Zierz, P. Joshi, M. Deschauer, 2020, Biomedicines.

Two novel mutations in the myophosphorylase gene in a patient with McArdle disease

S. Zierz, M. Deschauer, K. Hertel, 2003, Muscle & nerve.

[Postpartum obturator nerve syndrome: case report and review of the nerve compression syndrome during pregnancy and delivery].

A. Lindner, S. Zierz, W. Schulte-Mattler, 1997, Zentralblatt fur Gynakologie.

Only subtle cognitive deficits in non–bulbar amyotrophic lateral sclerosis patients

Albert C. Ludolph, Stephan Zierz, Bernd Leplow, 2006, Journal of Neurology.

An intronic base alteration of the CHRNE gene leading to a congenital myasthenic syndrome

Hanns Lochmüller, A. Huebner, R. Stucka, 2005, Neurology.

Reply to 'Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies--a comment'.

A. Krasnianski, M. Krasnianski, S. Zierz, 2006, Brain : a journal of neurology.

Unsupervised Classification of EEG Background Activity for Presurgical Epilepsy Diagnostics

M. Spreng, W. G. Hofmann, S. Zierz, 1997, DAGM-Symposium.

Prevalence of Pompe disease in 3,076 patients with hyperCKemia and limb-girdle muscular weakness

B. Schlotter-Weigel, B. Schoser, Z. Lukacs, 2016, Neurology.

Phenotype of carnitine palmitoyltransferase II (CPT II) deficiency: A questionnaire-based survey

S. Zierz, P. Joshi, M. Deschauer, 2019, Journal of Clinical Neuroscience.

False-positive diagnosis of a single, large-scale mitochondrial DNA deletion by Southern blot analysis: the role of neutral polymorphisms.

Robert W. Taylor, A. Krasnianski, S. Zierz, 2004, Genetic testing.

Impaired energy metabolism in septic hearts of baboons: diminished activities of complex I and complex II of the mitochondrial respiratory chain: 95

K. Werdan, G. Durrieu, H. Redl, 1997 .

Assessment of physical fatigability and fatigue perception in myasthenia gravis

U. Menge, S. Zierz, B. Jordan, 2017, Muscle & nerve.

MTHFR and ACE Polymorphisms Do Not Increase Susceptibility to Migraine Neither Alone Nor in Combination

R. Lea, H. Kress, S. Zierz, 2016, Headache.

Myositis in Lewis rats induced by the superantigen Staphylococcal enterotoxin A

M. Kornhuber, K. Delank, S. Zierz, 2019, Molecular Biology Reports.

Mutations of the Mitochondrial DNA: The Contribution of DNA Techniques to the Diagnosis of Mitochondrial Encephalomyopathies

K. Gerbitz, D. Pongratz, T. Deufel, 1990, Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie.

Function of the mitochondrial outer membrane as a diffusion barrier in health and diseases.

C. Neuhoff, S. Zierz, E. Seppet, 2000, Biochemical Society transactions.

Discharge patterns of spontaneous activity and motor units on concentric needle electromyography

S. Zierz, W. Schulte-Mattler, Dimitrios Georgiadis, 2001, Muscle & nerve.

Peripheral neuropathy in patients with CPEO associated with single and multiple mtDNA deletions

Robert W. Taylor, M. Kornhuber, A. Wienke, 2016, Neurology: Genetics.

Phenotype variability and histopathological findings in centronuclear myopathy due to DNM2 mutations

A. Dietz, J. Weis, W. Kress, 2011, Journal of Neurology.

Clinical features and differential diagnosis of flail arm syndrome

S. Petri, A. Ludolph, K. Kollewe, 2016, Journal of Neurology.

Puusepp's sign- clinical significance of a forgotten pyramidal sign

M. Krasnianski, S. Zierz, P. Tacik, 2008 .

Hirayama disease is a pure spinal motor neuron disorder—a combined DTI and transcranial magnetic stimulation study

J. Kaufmann, M. Kornhuber, A. Münchau, 2013, Journal of Neurology.

[Lactate and pyruvate blood levels in the diagnosis of mitochondrial myopathies].

S. Zierz, F. Jerusalem, S. Meessen, 1989, Der Nervenarzt.

Lactate production upon short-term non–ischemic forearm exercise in mitochondrial disorders and other myopathies

F. Hanisch, S. Zierz, K. Eger, 2006, Journal of Neurology.

Persistent ocular motor disturbances in migraine without aura

S. Zierz, T. Wieser, R. Wolff, 2004, Neurological Sciences.

Limited trypsin proteolysis renders carnitine palmitoyltransferase insensitive to inhibition by malonyl-CoA in patients with muscle carnitine palmitoyltransferase deficiency

S. Zierz, 1994, The clinical investigator.

Successful treatment of orbital myositis with intravenous immunoglobulins

A. Lindner, S. Zierz, Sarangerel Shambal, 1998, Muscle & nerve.

Pure myopathy associated with a novel mitochondrial tRNA gene mutation

D. Turnbull, S. Zierz, H. Swalwell, 2006, Neurology.

[Rhabdomyolysis and myoglobinuria].

A. Lindner, S. Zierz, 2003, Der Nervenarzt.

Cognitive fatigue in patients with myasthenia gravis

U. Menge, S. Zierz, B. Jordan, 2017, Muscle & nerve.

Doppler microembolic signals in patients with two different types of bileaflet valves.

S. Zierz, G. M. Bernacca, D. Georgiadis, 2001, The Journal of thoracic and cardiovascular surgery.

Occurrence of adrenocortical insufficiency in adrenomyeloneuropathy

S. Zierz, W. Kohler, W. Brennemann, 1996, Neurology.

Assay of acyl-CoA dehydrogenases in muscle and liver and identification of four new cases of medium-chain acyl-CoA dehydrogenase deficiency associated with systemic carnitine deficiency.

A. Engel, S. Zierz, C. Romshe, 1988, Advances in neurology.

Response: Late-onset Pompe disease manifests in the brain

S. Zierz, I. Schneider, O. Hensel, 2019, Molecular genetics and metabolism reports.

The p.P56S mutation in the VAPB gene is not due to a single founder: the first European case

P. Trillenberg, M. Zatz, J. Weis, 2010, Clinical genetics.

[Polymyositis associated with thymoma].

S. Zierz, K. Eger, B. Jordan, 2009, Der Nervenarzt.

Length dependence of variables associated with temporal dispersion in human motor nerves

M. Kornhuber, S. Zierz, D. Georgiadis, 2001, Muscle & nerve.

Cyclic vertical deviation after ocular myositis and treatment by recession of the inferior rectus muscle

S. Zierz, M. Sievert, V. Bau, 2005, Graefe's Archive for Clinical and Experimental Ophthalmology.

GFPT1-Associated Congenital Myasthenic Syndrome Mimicking a Glycogen Storage Disease - Diagnostic Pitfalls in Myopathology Solved by Next-Generation-Sequencing.

T. Haack, G. Stoltenburg‐Didinger, S. Zierz, 2022, Journal of neuromuscular diseases.

Recurrent Myalgia since Early Infancy—Misleading Clinical Course in a Child with Carnitine Palmitoyltransferase-II Deficiency

U. Ceglarek, S. Zierz, A. Merkenschlager, 2019, Neuropediatrics.

Switch to double positive late onset MuSK myasthenia gravis following thymomectomy in paraneoplastic AChR antibody positive myasthenia gravis

S. Zierz, B. Jordan, S. Schilling, 2015, Journal of Neurology.

MuSK–antibody positive pure ocular myasthenia gravis

F. Hanisch, S. Zierz, K. Eger, 2006, Journal of Neurology.

Chronic Idiopathic Intestinal Pseudo-obstruction

A. L. Wong, J. Belmont, M. Dichgans, 2011 .

[Cutaneous amyloidoses].

A. L. Wong, J. Belmont, D. Roden, 1990, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete.

[Chronic venous insufficiency].

V Wienert, G Hauer, A Lentner, 1996, Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen.

Successful use of an immune checkpoint inhibitor in a patient with myasthenia gravis in remission

S. Zierz, K. Jordan, B. Jordan, 2019, Muscle and Nerve.

Klassische alternierende Medulla-oblongata-Syndrome

M. Krasnianski, S. Zierz, M. Winterholler, 2003 .

Doppler microembolic signals in children with prosthetic cardiac valves.

A. Lindner, S. Zierz, D. Georgiadis, 1997, Stroke.

The ATS valve in children

A. Lindner, S. Zierz, D. Georgiadis, 1998 .

FV 3 Functional transcranial Doppler (fTCD) during cold-induced pain in the oral cavity and ice cream headache

T. Kraya, S. Zierz, O. Hensel, 2017, Clinical Neurophysiology.

Carnitine Acyltransferases Are Not Changed in Alzheimer Disease

S. Zierz, I. Maurer, H. Möller, 1998, Alzheimer disease and associated disorders.

[Differential sciatica pain diagnosis from the neurologic viewpoint].

A. Lindner, S. Zierz, 1997, Medizinische Klinik.

Clinical features and differential diagnosis of flail arm syndrome

S. Petri, A. Ludolph, K. Kollewe, 2016, Journal of Neurology.

Epidural Hematoma and Hemorrhagic Infarction Mimicking a Left Temporal Meningioma

W. Hacke, J. Baron, S. Schwab, 1999, Cerebrovascular Diseases.

“Patients with amyotrophic lateral sclerosis (ALS) are usually nice persons”—How physicians experienced in ALS see the personality characteristics of their patients

S. Zierz, B. Jordan, T. Mehl, 2016, Brain and behavior.

Corticomotoneuronal latencies after non-invasive stimulation of the motor cortex in adrenomyeloneuropathy and adrenoleukodystrophy: results in patients and their relatives.

C. Elger, A. Ludolph, H. Masur, 1990, Functional neurology.

PB 3 Abnormal height of basilar artery bifurcation in adults with Pompe disease

S. Zierz, I. Schneider, O. Hensel, 2017, Clinical Neurophysiology.

EP 42. Smooth muscle involvement in Glycogenosis Type II: Thalamus hemorrhage, increased blood flow and dolichoectasia in a late-onset Pompe patient

S. Zierz, I. Schneider, O. Hensel, 2016, Clinical Neurophysiology.

Neuromyelitis optica: eine mit Aquaporin-4-Antikörpern assoziierte nosologische Entität

S. Zierz, K. Eger, M. Kornhuber, 2008, Fortschritte der Neurologie-Psychiatrie.

Normal protein content but abnormally inhibited enzyme activity in muscle carnitine palmitoyltransferase II deficiency

S. Zierz, D. Lehmann, 2014, Journal of Neurological Sciences.

White matter lesions in treated late onset Pompe disease are not different to matched controls.

S. Zierz, I. Schneider, O. Hensel, 2019, Molecular genetics and metabolism.

Polyglucosan myopathy and functional characterization of a novel GYG1 mutation

T. Kraya, K. Visuttijai, A. Oldfors, 2017, Acta neurologica Scandinavica.

Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.

J. Dreyhaupt, J. Kassubek, A. Ludolph, 2016, Brain : a journal of neurology.

Lambert-Eaton-Myastheniesyndrom

S. Zierz, B. Jordan, 2020, Klinische Neurologie.

Lambert-Eaton-Myastheniesyndrom (LEMS)

S. Zierz, B. Jordan, 2020 .