Hlcs Gene Summary [Mouse]
Enables biotin--[biotin carboxyl-carrier protein] ligase activity. Involved in biotin metabolic process. Located in mitochondrion. Is expressed in several structures, including genitourinary system; gut; nervous system; sensory organ; and thymus. Human ortholog(s) of this gene implicated in holocarboxylase synthetase deficiency. Orthologous to human HLCS (holocarboxylase synthetase). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Hlcs |
| Official Name | holocarboxylase synthetase (biotin- [propriony-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase) [Source:MGI Symbol;Acc:MGI:894646] |
| Ensembl ID | ENSMUSG00000040820 |
| Bio databases IDs | |
| Aliases | holocarboxylase synthetase (biotin- |
| Synonyms | 410I21.SP6, D16Jhu34, HCS, holocarboxylase synthetase, holocarboxylase synthetase (biotin- [propriony-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase), RGD1565360 |
| 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 Hlcs often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- biotin binding
- birA, biotin-[acetyl-CoA-carboxylase] ligase region
- enzyme binding
- Biotin-protein ligase, N terminal
- enzyme
- protein binding
- identical protein binding
- biotin-[methylmalonyl-CoA-carboxyltransferase] ligase
- miscellaneous carbon-nitrogen ligase
- biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase
- biotin-lipoate ligase family
- biotin-[acetyl-CoA-carboxylase] ligase
- Biotin protein ligase C terminal domain
- biotin-[methylcrotonoyl-CoA-carboxylase] ligase
Pathways
Biological processes and signaling networks where the Hlcs 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
- COVID-19
- major depression
- holocarboxylase synthetase deficiency
- hereditary disorder
- specific language impairment
- glioblastoma
- glioblastoma cancer
- bipolar disorder
- eosinophilic esophagitis
- cerebral aneurysm
role in cell
- upregulation in
- downregulation 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
- Mitochondria
- cytosol
- nuclear matrix
- nuclear lamina
- chromatin
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Hlcs gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- biotin metabolic process
- response to biotin
- post-translational protein modification
Cellular Component
Where in the cell the gene product is active
- cytoplasm
- mitochondrion
- nuclear matrix
- cytosol
- chromatin
- nuclear lamina
Molecular Function
What the gene product does at the molecular level
- ATP binding
- identical protein binding
- biotin-protein ligase activity
- biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity
- protein binding
- biotin binding
- biotin-[methylcrotonoyl-CoA-carboxylase] ligase activity
- biotin-[acetyl-CoA-carboxylase] ligase activity
- biotin-[methylmalonyl-CoA-carboxytransferase] ligase activity
- enzyme binding