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NEDCASB

Chr 12AR

serine hydroxymethyltransferase 2

Also known as: GLYA, HEL-S-51e, NEDCASB, SHMT, mSHMT

This gene encodes the mitochondrial form of serine hydroxymethyltransferase, a pyridoxal phosphate-dependent enzyme that catalyzes the conversion of serine and tetrahydrofolate to glycine and 5,10-methylene tetrahydrofolate and serves as the primary source of intracellular glycine synthesis. Biallelic mutations cause an autosomal recessive neurodevelopmental disorder with cardiomyopathy, spasticity, and brain abnormalities. This multisystem disorder affects both the central nervous system and cardiac function, reflecting the critical role of glycine metabolism in cellular development and function.

OMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/NEDCASB?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NEDCASB · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

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