Fh1 Gene Summary [Mouse]
Enables fumarate hydratase activity. Involved in several processes, including carboxylic acid metabolic process; positive regulation of cold-induced thermogenesis; and tricarboxylic acid cycle. Acts upstream of or within homeostasis of number of cells within a tissue. Located in mitochondrion. Is active in mitochondrial matrix. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in fumarase deficiency and lung non-small cell carcinoma. Orthologous to human FH (fumarate hydratase). [provided by Alliance of Genome Resources, Apr 2025]
Details
| Type | Protein Coding |
| Official Symbol | Fh1 |
| Official Name | fumarate hydratase 1 [Source:MGI Symbol;Acc:MGI:95530] |
| Ensembl ID | ENSMUSG00000026526 |
| Bio databases IDs | |
| Aliases | fumarate hydratase 1 |
| Synonyms | Fh1, FMRD, Fumarase, Fumarate Hydratase, fumarate hydratase 1, Fumh, HLRCC, HsFH, LRCC, MCL, MCUL1, Mitochondrial Fumarase |
| 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 Fh1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- histone binding
- aspartate ammonia-lyase
- fumarate hydratase, class II
- argininosuccinate lyase
- fumarate hydratase
- Fumarase C C-terminus
- Lyase class I-like
- lyase
- enzyme
- protein binding
Pathways
Biological processes and signaling networks where the Fh1 gene in mouse plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- liver neoplasia
- epithelial neoplasia
- neoplasia
- liver cancer
- digestive system cancer
- abdominal cancer
- cancer
- epithelial cancer
- hypertension
- proteinuria
role in cell
- expression in
- apoptosis
- transmembrane potential
- growth
- cell viability
- proliferation
- quantity
- phosphorylation in
- migration
- production in
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
- membrane fraction
- Nucleus
- Mitochondria
- cytosol
- synaptic membrane
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Fh1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- DNA repair
- malate metabolic process
- homeostasis of number of cells within a tissue
- tricarboxylic acid cycle
- fumarate metabolic process
Cellular Component
Where in the cell the gene product is active
- nucleus
- extracellular vesicular exosome
- cytoplasm
- mitochondrial matrix
- mitochondrion
- cytosol
- chromosome
Molecular Function
What the gene product does at the molecular level
- protein binding
- fumarate hydratase activity