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ACADVL, Recombinant, Human (Acyl Coenzyme A Dehydrogenase Very Long Chain, Acyl-CoA Dehydrogenase Very Long Chain, ACAD6, LCACD, Very Long-chain Specific Acyl-CoA Dehydrogenase Mitochondrial, VLCAD)

Cat no: A0193-31

ACADVL, Recombinant, Human (Acyl Coenzyme A Dehydrogenase Very Long Chain, Acyl-CoA Dehydrogenase Very Long Chain, ACAD6, LCACD, Very Long-chain Specific Acyl-CoA Dehydrogenase Mitochondrial, VLCAD)

ACADVL, also known as VLCAD, LCACD or ACAD6, is an inner mitochondrial membrane protein that belongs to the family of acyl-CoA dehydrogenases. This protein is involved in lipid metabolism and has catalytic activity toward esters of long chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA, and functions in the first step of the fatty acid b-oxidation pathway. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.\n\nSource:\nRecombinant corresponding to aa41-655 of human ACADVL protein, fused to His-tag at N-terminus, expressed in E.coli.\n\nAA Sequence:\nMGSSHHHHHH SSGLVPRGSH MAGGAAQLAL DKSDSHPSDA LTRKKPAKAE SKSFAVGMFK GQLTTDQVFP YPSVLNEEQT QFLKELVEPV SRFFEEVNDP AKNDALEMVE ETTWQGLKEL GAFGLQVPSE LGGVGLCNTQ YARLVEIVGM HDLGVGITLG AHQSIGFKGI LLFGTKAQKE KYLPKLASGE TVAAFCLTEP SSGSDAASIR TSAVPSPCGK YYTLNGSKLW ISNGGLADIF TVFAKTPVTD PATGAVKEKI TAFVVERGFG GITHGPPEKK MGIKASNTAE VFFDGVRVPS ENVLGEVGSG FKVAMHILNN GRFGMAAALA GTMRGIIAKA VDHATNRTQF GEKIHNFGLI QEKLARMVML QYVTESMAYM VSANMDQGAT DFQIEAAISK IFGSEAAWKV TDECIQIMGG MGFMKEPGVE RVLRDLRIFR IFEGTNDILR LFVALQGCMD KGKELSGLGS ALKNPFGNAG LLLGEAGKQL RRRAGLGSGL SLSGLVHPEL SRSGELAVRA LEQFATVVEA KLIKHKKGIV NEQFLLQRLA DGAIDLYAMV VVLSRASRSL SEGHPTAQHE KMLCDTWCIE AAARIREGMA ALQSDPWQQE LYRNFKSISK ALVERGGVVT SNPLGF\n\nMolecular Weight: 68.5kD (636aa) confirmed by MALDI-TOF\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

A0193-31

Size

50ug

Form

Supplied as a liquid in 20mM Tris-HCl, pH 8.0, 10% glycerol, 1mM DTT, 1mM EDTA, 0.1M sodium chloride.

Purity

~90% (SDS-PAGE).

References

1. Smelt AH., et al. (1998) Ann Neurol. 43(4):540-4. 2. Souri M., et al. (1996) Am J Hum Genet. 58(1):97-106.

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