Gamma-butyrobetaine dioxygenase
| BBOX1 | |||
|---|---|---|---|
Gene ontology | |||
| Molecular function |
| ||
| Cellular component | |||
| Biological process | |||
| Sources:Amigo / QuickGO | |||
Ensembl | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| UniProt | |||||||||
| RefSeq (mRNA) | |||||||||
| RefSeq (protein) | |||||||||
| Location (UCSC) | Chr 11: 27.04 – 27.13 Mb | Chr 2: 110.09 – 110.14 Mb | |||||||
| PubMed search | [3] | [4] | |||||||
| View/Edit Human | View/Edit Mouse |
Gamma-butyrobetaine dioxygenase (also known as BBOX, GBBH or γ-butyrobetaine hydroxylase) is an
Reaction
| gamma-butyrobetaine dioxygenase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
Gamma-butyrobetaine dioxygenase belongs to the
- 4-trimethylammoniobutanoate (γ-butyrobetaine) + 2-oxoglutarate+ O2 3-hydroxy-4-trimethylammoniobutanoate (succinate+ CO2
The three
This enzyme belongs to the family of
Both the apo (PDB id: 3N6W)
Gamma-butyrobetaine dioxygenase is
Inhibition
Gamma-butyrobetaine dioxygenase is an inhibition target for 3-(2,2,2-trimethylhydraziniumyl)propionate (
Mildronate has a similar structure to the natural substrate gamma-butyrobetaine, with a NH group replacing the CH2 of gamma-butyrobetaine at the C-4 position. A crystal structure of mldronate in complex with gamma-butyrobetaine dioxygenase was published, and it suggests mildronate bind to gamma-butyrobetaine dioxygenase in exactly the same way as gamma-butyrobetaine (PDB id: 3MS5).
Similar to other
Assay
Several
See also
- Carnitine
- Mildronate
- Carnitine biosynthesis
- Beta Oxidation
- Fatty acid metabolism
- Stevens rearrangement
References
- ^ a b c GRCh38: Ensembl release 89: ENSG00000129151 – Ensembl, May 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000041660 – Ensembl, May 2017
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ PMID 9753662.
- ^ a b "Entrez Gene: BBOX1 butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1".
- PMID 1735445.
- PMID 7156861.
- S2CID 24740881.
- PMID 4396068.
- PMID 1962562.
- PMID 6783120.
- PMID 1962562.
- PMID 18758058.
- PMID 3378057.
- PMID 20599753.
- ^ PMID 21168767.
- ^ PMID 21168760.
- PMID 3342076.
- ^ PMID 22765904.
- .
- PMID 18277970.
- S2CID 45844835.
- S2CID 1812127.
- PMID 10812052.
- PMID 16007237.
- S2CID 38603504.
- PMID 21371472.
- S2CID 12501700.
- ISSN 1648-7966. Archived from the original(PDF) on 2012-03-28.
- ^ Vitols A, Voita D, Dzerve V (2008). "Mildronate improves carotid baroreceptor reflex function in patients with chronic heart failure" (PDF). Semin Cardiovasc Med. 13: 6. Archived from the original (PDF) on 2013-09-21.
- ^ Dzerve V, Matisone D, Pozdnyakov Y, Oganov R (2010). "Mildronate improves the exercise tolerance in patients with stable angina: results of a long term clinical trial" (PDF). Semin Cardiovasc Med. 16: 3. Archived from the original (PDF) on 2013-09-21.
- PMID 22186118.
- S2CID 697191.
- ^ "Tennis pro Maria Sharapova says she takes 'full responsibility' for failed drug test". LA Times. 8 March 2016.
- ^ PDB: 3MS5
- PMID 21390379.
- S2CID 23339575.
- PMID 11248709.
- PMID 1988434.
- .
- .
- PMID 5472709.
- PMID 861203.
- PMID 5022087.
- PMID 4279150.
- PMID 869176.
- S2CID 13956474.
- PMID 19436879.
Further reading
- Olson AL, Rebouche CJ (Jun 1987). "gamma-Butyrobetaine hydroxylase activity is not rate limiting for carnitine biosynthesis in the human infant". The Journal of Nutrition. 117 (6): 1024–31. PMID 3110383.
- Lindstedt S, Nordin I (Oct 1984). "Multiple forms of gamma-butyrobetaine hydroxylase (EC 1.14.11.1)". The Biochemical Journal. 223 (1): 119–27. PMID 6497835.
- Galland S, Le Borgne F, Bouchard F, Georges B, Clouet P, Grand-Jean F, Demarquoy J (Oct 1999). "Molecular cloning and characterization of the cDNA encoding the rat liver gamma-butyrobetaine hydroxylase". Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids. 1441 (1): 85–92. PMID 10526231.
- Rigault C, Le Borgne F, Demarquoy J (Dec 2006). "Genomic structure, alternative maturation and tissue expression of the human BBOX1 gene". Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids. 1761 (12): 1469–81. PMID 17110165.
