protein

ACADVL, 41-655aa, Human, His tag, E Coli

MBS203547

0.05 mg

375 USD

Recombinant Protein

ACADVL, 41-655aa, Human, His tag, E Coli

ACADVL

ACAD6; LCACD; VLCAD; Very long-chain specific acyl-CoA dehydrogenase; mitochondrial

very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1; Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; very long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, very long chain; acyl-CoA dehydrogenase, very long chain

ACADVL

ACADVL; ACADVL; ACAD6; LCACD; VLCAD; VLCAD; VLCAD

ACADV_HUMAN

655

MGSSHHHHHH 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

> 90 % by SDS - PAGE

Liquid. In 20mM Tris-HCl buffer(pH 8.0) containing 10% glycerol 1mM DTT, 1mM EDTA, 0.1M NaCl.

0.5 mg/ml (determined by Bradford assay)

Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.

SDS-PAGE

Antigen Species: Human. Tag: His-tag

Expression System: E Coli

Enzymes & Proteases

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 beta-oxidation pathway. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Recombinant human ACADVL protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

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

4557235

NP_000009

NM_000018.3

68.5 kDa (636aa)

Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway (106124); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084); Fatty Acid Metabolism Pathway (878045); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); IRE1alpha Activates Chaperones Pathway (105906); Metabolic Pathways (132956)

The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

ACADVL: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Oxidoreductase; EC 1.3.8.9; Lipid Metabolism - fatty acid; Mitochondrial. Chromosomal Location of Human Ortholog: 17p13.1. Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm; mitochondrial inner membrane; nucleolus; nucleus. Molecular Function: acyl-CoA dehydrogenase activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity. Biological Process: cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; energy derivation by oxidation of organic compounds. Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of

0.05 mg

375 USD

0.25 mg

925