BBOX1, also known as gamma-BBH, belongs to the gamma-BBH/TMLD family. It is highly expressed in kidney and moderately expressed in liver. BBOX1 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 beta-oxidation. BBOX1 is an inhibition target for mildronate which can be used to treat angina andmyocardial infarction. Mildronate may also be beneficial for the treatment ofneurological disorder, diabetes, and seizures and alcohol intoxication.
Product Name:
Human BBOX1/Gamma-BBH Recombinant Protein (RPES3536)
Product Code:
RPES3536
Size:
100 µg
Species:
Human
Expressed Host:
Baculovirus-Insect Cells
Synonyms:
BBH,BBOX,G-BBH,gamma-BBH
Accession:
O75936
Sequence:
Met 1-Asn387
Fusion tag:
N-His & GST
Endotoxin:
<1.0 EU per µg as determined by the LAL method.
Protein Construction:
A DNA sequence encoding the human BBOX1 (O75936) (Met1-Asn387) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
This product is provided as lyophilized powder which is shipped with ice packs.
Stability and Storage:
Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
