protein

Recombinant Human Acyl-CoA Dehydrogenase, Very Long Chain

MBS144138

0.002 mg

140 USD

Recombinant Protein

Recombinant Human Acyl-CoA Dehydrogenase, Very Long Chain

Acyl-CoA Dehydrogenase

ACADVL Human; Acyl-CoA Dehydrogenase, Very Long Chain Human Recombinant; ACAD6; LCACD; VLCAD

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

E Coli

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.

Greater than 90.0% as determined by SDS-PAGE.

The 0.5mg/ml protein solution contains 20mM Tris-HCl buffer pH-8, 1mM DTT, 1mM EDTA, 10% glycerol and 100mM NaCl. Sterile Filtered clear solution.

Store ACADVL at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

ENZYMES; Enzymes; Dehydrogenase

Description: ACADVL Human Recombinant produced in E Coli is a non-glycosylated, polypeptide chain containing 636 amino acids (41-655 a.a.) and having a molecular mass of 68.5 kda. ACADVL contains 21 amino acid His-Tag at the N-terminus and is purified by proprietary chromatographic techniques. Introduction: ACADVL is an inner mitochondrial membrane enzyme that is part of the family of acyl-CoA dehydrogenases. ACADVL protein participates 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 is involved in the first step of the fatty acid beta-oxidation pathway. ACADVL deficiency in reduces myocardial fatty acid beta-oxidation and is related with cardiomyopathy.

4557235

NP_000009.1

NM_000018.3

P49748

72,927 Da

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; mitochondrial inner membrane; cytoplasm; nucleolus; nucleus. Molecular Function: acyl-CoA dehydrogenase activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity. Biological Process: unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; 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

0.002 mg

140 USD

0.01 mg

205

1 mg

5015