Gcsh Gene Summary [Rat]

Enables aminomethyltransferase activity and enzyme binding activity. Involved in glycine decarboxylation via glycine cleavage system. Predicted to be part of glycine cleavage complex. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in multiple mitochondrial dysfunctions syndrome. Orthologous to human GCSH (glycine cleavage system protein H). [provided by Alliance of Genome Resources, Jul 2025]

Details

Type
Protein Coding
Official Symbol
Gcsh
Official Name
glycine cleavage system protein H [Source:RGD Symbol;Acc:619946]
Ensembl ID
ENSRNOG00000011535
Bio databases IDs NCBI: 171133 Ensembl: ENSRNOG00000011535
Aliases glycine cleavage system protein H
Synonyms 1100001L02Rik, 5730591C18RIK, GCE, glycine cleavage system protein H, glycine cleavage system protein H (aminomethyl carrier), MMDS7, NKH
Species
Rat, Rattus norvegicus
OrthologiesHumanMouse

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in rat Gcsh often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • Biotinyl_lipoyl_domains
  • aminomethyltransferase
  • enzyme binding
  • enzyme
  • protein binding

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • multiple mitochondrial dysfunctions syndrome type 7
  • urothelial bladder carcinoma
  • transitional cell bladder cancer
  • non-ketotic hyperglycinemia
  • nonalcoholic fatty liver disease
  • infection by Hepatitis C virus genotype 2
regulated by
regulates
  • Glycine cleavage system
  • glycine
role in cell
  • accumulation in
  • accumulation

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • Mitochondria
  • mitochondrial matrix
  • plasma

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the rat Gcsh gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • glycine decarboxylation via glycine cleavage system
  • glycine catabolic process
  • protein lipoylation

Cellular Component

Where in the cell the gene product is active
  • cytoplasm
  • mitochondrial matrix
  • mitochondrion
  • glycine cleavage complex

Molecular Function

What the gene product does at the molecular level
  • protein binding
  • aminomethyltransferase activity