ACADVL blocking peptide

ACADVL Peptide - C-terminal region

Cat. Num. AAA3236644

• Gene Names: ACADVL; ACAD6; LCACD; VLCAD
• Reactivity: Human
• Applications: Western Blot
• Synonyms: ACADVL; ACADVL Peptide - C-terminal region; ACADVL blocking peptide

• Reactivity: Human
• Form/Format: Lyophilized powder
• Sequence: IDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQ
• Sequence Length: 633

• Applicable Applications for ACADVL blocking peptide: Western Blot (WB)
• Preparation and Storage: Add 100ul of sterile PBS. Final peptide concentration is 1 mg/ml in PBS. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.

Related Product Information for ACADVL blocking peptide

This is a synthetic peptide designed for use in combination with anti-ACADVL Antibody, made

Target Description: ACADVL 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 ACADVL protein reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.

Product Categories/Family for ACADVL blocking peptide

Peptide

NCBI and Uniprot Product Information

• NCBI GI #: 76496475
• NCBI GeneID: 37
• NCBI Accession #: NP_001029031
• NCBI GenBank Nucleotide #: NM_001033859
• UniProt Accession #: P49748
• Molecular Weight: 64kDa
• NCBI Official Full Name: very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase very long chain
• NCBI Official Symbol: ACADVL
• NCBI Official Synonym Symbols: ACAD6; LCACD; VLCAD
• NCBI Protein Information: very long-chain specific acyl-CoA dehydrogenase, mitochondrial
• UniProt Protein Name: Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
• Protein Family: Very long-chain specific acyl-CoA dehydrogenase
• UniProt Gene Name: ACADVL
• UniProt Synonym Gene Names: VLCAD; VLCAD
• UniProt Entry Name: ACADV_HUMAN

NCBI Description

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]

Uniprot Description

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