Gcsh Gene Summary [Mouse]
Predicted to enable aminomethyltransferase activity and enzyme binding activity. Predicted to be involved in glycine decarboxylation via glycine cleavage system and protein lipoylation. Located in mitochondrion. Is expressed in several structures, including alimentary system; integumental system; metanephros; nervous system; and respiratory system. 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, Apr 2025]
Details
| Type | Protein Coding |
| Official Symbol | Gcsh |
| Official Name | glycine cleavage system protein H (aminomethyl carrier) [Source:MGI Symbol;Acc:MGI:1915383] |
| Ensembl ID | ENSMUSG00000034424 |
| Bio databases IDs | |
| Aliases | glycine cleavage system protein H (aminomethyl carrier) |
| Synonyms | 1100001L02Rik, 5730591C18RIK, GCE, glycine cleavage system protein H, glycine cleavage system protein H (aminomethyl carrier), MMDS7, NKH |
| Species | Mouse, Mus musculus |
| Orthologies |
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 mouse 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
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 mouse 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