About

Jeremy Schneider, M.D., is an Associate Clinical Professor in the Department of Dermatology at UC San Diego. His research and clinical work focus on dermatological conditions, including immune checkpoint inhibitor-induced toxic epidermal necrolysis, Stevens-Johnson syndrome, toxic epidermal necrolysis, and other dermatological manifestations associated with systemic diseases. Dr. Schneider has contributed to numerous publications in the field of dermatology, with research spanning topics such as pharmacogenomics, dermatological syndromes, and treatment strategies for complex skin conditions. His work involves both clinical case reports and reviews, emphasizing the management and understanding of severe dermatological reactions and syndromes.

Research topics

• Medicine
• Chemistry
• Internal medicine
• Biochemistry
• Endocrinology

Selected publications

• A Personal History of Cystinosis by Dr. Jerry Schneider

  Cells · 2022-03-10 · 1 citations

  editorialOpen access1st author

  Cystinosis is a rare lysosomal storage disease that is tightly linked with the name of the American physician and scientist Dr. Jerry Schneider. Dr. Schneider (1937–2021) received his medical degree from Northwestern University, followed by a pediatrics residency at Johns Hopkins University and a fellowship in inherited disorders of metabolism. He started to work on cystinosis in J. Seegmiller’s laboratory at the National Institutes of Health (NIH) and subsequently moved to the UC San Diego School of Medicine, where he devoted his entire career to people suffering from this devastating lysosomal storage disorder. In 1967, Dr. Schneider’s seminal Science paper ‘Increased cystine in leukocytes from individuals homozygous and heterozygous for cystinosis’ opened a new era of research towards understanding the pathogenesis and finding treatments for cystinosis patients. His tremendous contribution transformed cystinosis from a fatal disorder of childhood to a treatable chronic disease, with a new generation of cystinosis patients being now in their 40th and 50th years. Dr. Schneider wrote a fascinating ‘Personal History of Cystinosis’ highlighting the major milestones of cystinosis research. Unfortunately, he passed away before this manuscript could be published. Fifty-five years after his first paper on cystinosis, the ‘Personal History of Cystinosis’ by Dr. Schneider is a tribute to his pioneering discoveries in the field and an inspiration for young doctors and scientists who have taken over the torch of cystinosis research towards finding a cure for cystinosis.

  PublisherOA PDFDOI

• Controversies and research agenda in nephropathic cystinosis: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

  Kidney International · 2016-05-12 · 81 citations

  article
  PublisherDOI

• Nephropathic Cystinosis

  2015-04-20

  book-chapterSenior author
  PublisherDOI

• Copper Deficiency in Patients with Cystinosis with Cysteamine Toxicity

  The Journal of Pediatrics · 2013-05-04 · 40 citations

  article
  PublisherDOI

• Copper deficiency in cystinosis patients

  Pediatric Nephrology · 2012-09-01 · 2 citations

  article
  Publisher

• Cysteamine Toxicity in Patients with Cystinosis

  The Journal of Pediatrics · 2011-07-26 · 78 citations

  article
  PublisherDOI

• Pharmacokinetics of enteric‐coated cysteamine bitartrate in healthy adults: a pilot study

  British Journal of Clinical Pharmacology · 2010-06-08 · 27 citations

  articleOpen access

  WHAT IS ALREADY KNOWN ABOUT THIS SUBJECT: Cysteamine bitartrate is taken lifelong, every 6 h and for the treatment of cystinosis. Recent studies using cysteamine for for other diseases such as neurodegenerative disorders adopt the same dosing regimen for cysteamine. Regular cysteamine bitartrate (Cystagon) may cause upper gastrointestinal symptoms in some patients. WHAT THIS STUDY ADDS: This is the only study that provides pharmacokinetic data for cysteamine delivered in an enteric-release preparation in normal subjects. EC-cysteamine is very well tolerated and does not cause increased gastrin concentrations, even at relatively high doses. EC-cysteamine at the higher dose results in better drug uptake as measured by Cmax and AUC and is more likely to be effective. AIMS: Cysteamine bitartrate (Cystagon) is the approved treatment for cystinosis. Poor compliance and patient outcome may occur because the drug needs to be taken every 6 h and in some patients causes gastrointestinal symptoms due to hypergastrinaemia. A formulation of cysteamine requiring twice daily ingestion would improve the quality of life for these patients. This study compares the pharmacokinetics and gastrin production following cysteamine bitartrate non-enteric-coated and cysteamine bitartrate enteric-coated in normal healthy subjects. METHODS: Enteric-coated cysteamine was prepared. Following single doses of cysteamine bitartrate non-enteric-coated 450 mg and cysteamine bitartrate enteric-coated 450 mg and 900 mg, serial plasma cysteamine and gastrin concentrations were measured. Two subjects also received cysteamine bitartrate non-enteric-coated 900 mg. Gastrointestinal (GI) symptoms were recorded. RESULTS: Six healthy adults (mean age 20.7 years, range 18-24 years; mean weight 59.3 kg) received drug. All post-dose gastrin concentrations were within the normal range (<100 pg ml(-1)). The tmax following cysteamine bitartrate non-enteric-coated (mean and SD is 75+/-19 min) was shorter than cysteamine bitartrate enteric-coated (220+/-74 min) (P=0.001), but only the Cmax and AUC estimates following 900 mg cysteamine bitartrate enteric-coated were significantly greater than any of the other preparations or doses (P<0.05). One patient had GI symptoms following both 900 mg cysteamine bitartrate non-enteric-coated and cysteamine bitartrate enteric-coated. CONCLUSION: Although patient numbers were low, single high doses of cysteamine bitartrate enteric-coated were better tolerated than similar doses of cysteamine bitartrate non-enteric-coated in the healthy subjects and all had normal gastrin concentrations. The delayed tmax following cysteamine bitartrate enteric-coated suggested that the cysteamine was released enterically.

  PublisherOA PDFDOI

• Second International Cystinosis Research Symposium

  Pediatric Nephrology · 2010-10-01

  article1st authorCorresponding
  Publisher

• Long-Term Treatment of Cystinosis in Children with Twice-Daily Cysteamine

  The Journal of Pediatrics · 2010-02-09 · 28 citations

  article
  PublisherDOI

• High energy phosphotransfer in the failing mouse heart-role of adenylate kinase and glycolytic enzymes

  Oxford University Research Archive (ORA) (University of Oxford) · 2010-01-01

  article

  AIMS: To measure the activity of the key phosphotransfer enzymes creatine kinase (CK), adenylate kinase (AK), and glycolytic enzymes in two common mouse models of chronic heart failure. METHODS AND RESULTS: C57BL/6 mice were subjected to transverse aortic constriction (TAC), myocardial infarction induced by coronary artery ligation (CAL), or sham operation. Activities of phosphotransfer enzymes CK, AK, glyceraldehyde-3-phosphate dehydrogenase (GAPDH), 3-phosphoglycerate kinase (PGK), and pyruvate kinase were assessed spectrophotometrically. Mice were characterized by echocardiography or magnetic resonance imaging 5- to 8-week post-surgery and selected for the presence of congestive heart failure. All mice had severe left ventricular hypertrophy, impaired systolic function and pulmonary congestion compared with sham controls. A significant decrease in myocardial CK and maximal CK reaction velocity was observed in both experimental models of heart failure. However, the activity of AK and its isoforms remained unchanged, despite a reduction in its protein expression. In contrast, the activities of glycolytic phosphotransfer mediators GAPDH and PGK were 19 and 12% higher in TAC, and 31 and 23% higher in CAL models, respectively. CONCLUSION: Chronic heart failure in the mouse is characterized by impaired CK function, unaltered AK, and increased activity of glycolytic phosphotransfer enzymes. This pattern of altered phosphotransfer activity was observed independent of the heart failure aetiology.

  Publisher

Frequent coauthors

• Jess G. Thoene

  University of Michigan–Ann Arbor

  67 shared

• William A. Gahl

  61 shared

• Joseph D. Schulman

  California Department of Health Care Services

  35 shared

• J. Edwin Seegmiller

  29 shared

• Adam J. Jonas

  University of California, Los Angeles

  25 shared

• Kathryn H. Bradley

  24 shared

• Karen F Clark

  University of California, San Diego

  23 shared

• Margaret L. Smith

  University of California, San Diego

  23 shared

Education

• M.D.

  University of California, San Diego

  1995

• B.S.

  University of California, San Diego

  1991