| 619756 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits gamma-butyrobetaine dioxygenase activity; identical protein binding (ortholog); zinc ion binding (ortholog); INVOLVED IN carnitine biosynthetic process (ortholog); PARTICIPATES IN carnitine biosynthetic pathway; lysine degradation pathway; ASSOCIATED WITH Experimental Liver Cirrhosis (ortholog); FOUND IN cytosol (ortholog); INTERACTS WITH (+)-schisandrin B; 1-benzylpiperazine; 1-naphthyl isothiocyanate | 3 | 117277226 | 117326383 | Rat | 168 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 732171 | BBOX1 | gamma-butyrobetaine hydroxylase 1 | This gene encodes gamma butyrobetaine hydroxylase which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial bet a-oxidation. [provided by RefSeq, Jul 2008] | 11 | 27040815 | 27127809 | Human | 159 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 732172 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | Enables gamma-butyrobetaine dioxygenase activity. Involved in carnitine biosynthetic process. Located in mitochondrion. Is active in cytosol. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and retina. Orthologous to human ... (more)pan style='font-weight:700;'>BBOX1 (gamma-butyrobetaine hydroxylase 1). [provided by Alliance of Genome Resources, Jul 2025] | 2 | 110094401 | 110145158 | Mouse | 175 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8751103 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits gamma-butyrobetaine dioxygenase activity (ortholog); identical protein binding (ortholog); zinc ion binding (ortholog); INVOLVED IN carnitine biosynthetic process (ortholog); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrhosis (ortholog); FOUND IN cytosol (ortholog) | NW_004955476 | 6368842 | 6433797 | Chinchilla | 12 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12025492 | BBOX1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits dioxygenase activity (inferred); gamma-butyrobetaine dioxygenase activity (inferred); identical protein binding (inferred); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrho sis (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); mitochondrion (inferred) | 11 | 26995120 | 27083832 | Bonobo | 23 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12109766 | BBOX1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits identical protein binding (ortholog); zinc ion binding (ortholog); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrhosis (ortholog); FOUND IN cytosol (ortholog) | 21 | 47707648 | 47766732 | Dog | 20 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12627844 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits 2-oxoglutarate-dependent dioxygenase activity (inferred); dioxygenase activity (inferred); gamma-butyrobetaine dioxygenase activity (inferred); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Exper imental Liver Cirrhosis (ortholog); FOUND IN cytosol (inferred); mitochondrion (inferred) | NW_004936540 | 6048481 | 6113627 | Squirrel | 22 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 625976179 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits gamma-butyrobetaine dioxygenase activity (ortholog); identical protein binding (ortholog); zinc ion binding (ortholog); INVOLVED IN carnitine biosynthetic process (ortholog); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrhosis (ortholog); FOUND IN cytosol (ortholog) | | | | Black Rat | 12 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18913504 | Bbox1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits identical protein binding (ortholog); zinc ion binding (ortholog); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrhosis (ortholog); FOUND IN cytosol (ortholog) | | | | Naked Mole-Rat | 20 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155244965 | bbox1 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 18339842 | BBOX1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits dioxygenase activity (inferred); gamma-butyrobetaine dioxygenase activity (inferred); identical protein binding (inferred); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Experimental Liver Cirrho sis (ortholog); FOUND IN cytoplasm (inferred); cytosol (inferred); mitochondrion (inferred) | | | | Green Monkey | 23 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 13927718 | BBOX1 | gamma-butyrobetaine hydroxylase 1 | ENCODES a protein that exhibits 2-oxoglutarate-dependent dioxygenase activity (inferred); dioxygenase activity (inferred); gamma-butyrobetaine dioxygenase activity (inferred); INVOLVED IN carnitine biosynthetic process (inferred); PARTICIPATES IN carnitine biosynthetic pathway; ASSOCIATED WITH Exper imental Liver Cirrhosis (ortholog); FOUND IN cytosol (inferred); INTERACTS WITH deoxynivalenol | | | | Pig | 23 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155252359 | bbox1.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 8698737 | BBOX1-AS1 | BBOX1 antisense RNA 1 | INTERACTS WITH 2-hydroxypropanoic acid; aflatoxin B1; benzo[a]pyrene | 11 | 27047186 | 27220113 | Human | 9 | symbol , COSMIC , name , Human Proteome Map | gene, ncrna, VALIDATED [RefSeq] |
| 14700841 | LGR4-AS1 | LGR4 antisense RNA 1 | | 11 | 27471731 | 27482433 | Human | | old_gene_symbol | gene, ncrna, VALIDATED [RefSeq] |