Rabbit ACSF3 Polyclonal Antibody | anti-ACSF3 antibody

Anti-ACSF3 Antibody

Cat. Num. AAA8305946

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Purified by Immunogen Affinity Chromatography
• Synonyms: ACSF3; Polyclonal Antibody; Anti-ACSF3 Antibody; Acyl-CoA synthetase family member 3 mitochondrial; anti-ACSF3 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: Recognizes endogenous levels of ACSF3 protein.
• Purity/Purification: Purified by Immunogen Affinity Chromatography
• Form/Format: Liquid in 0.42% Potassium Phosphate, 0.87% Sodium Chloride, pH 7.3, 30% Glycerol, and 0.01% Sodium Azide.

• Applicable Applications for anti-ACSF3 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: Recombinant fusion protein of human ACSF3. The exact sequence is proprietary.
• Preparation and Storage: Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Western Blot (WB)

(Western blot analysis of ACSF3 expression in HEK293Tmouse heart (A), mouse kidney (B), rat brain (C) whole cell lysates.)

Related Product Information for anti-ACSF3 antibody

Rabbit polyclonal antibody to ACSF3

NCBI and Uniprot Product Information

• NCBI GI #: 187761343
• NCBI GeneID: 197322
• NCBI Accession #: NP_777577.2
• NCBI GenBank Nucleotide #: NM_174917.4
• UniProt Accession #: Q4G176; Q3URE1
• Molecular Weight: 64,130 Da
• NCBI Official Full Name: acyl-CoA synthetase family member 3, mitochondrial isoform 1
• NCBI Official Synonym Full Names: acyl-CoA synthetase family member 3
• NCBI Official Symbol: ACSF3
• NCBI Protein Information: acyl-CoA synthetase family member 3, mitochondrial; malonyl-CoA synthetase
• UniProt Protein Name: Acyl-CoA synthetase family member 3, mitochondrial
• Protein Family: Acyl-CoA synthetase family
• UniProt Gene Name: ACSF3
• UniProt Entry Name: ACSF3_HUMAN

NCBI Description

This gene encodes a member of the acyl-CoA synthetase family of enzymes that activate fatty acids by catalyzing the formation of a thioester linkage between fatty acids and coenzyme A. The encoded protein is localized to mitochondria, has high specificity for malonate and methylmalonate and possesses malonyl-CoA synthetase activity. Mutations in this gene are a cause of combined malonic and methylmalonic aciduria. Alternatively spliced transcript variants have been observed for this gene. [provided by RefSeq, Sep 2013]

Uniprot Description

Function: Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates. Ref.6 Ref.8

Subcellular location: Mitochondrion Ref.8.

Involvement in disease: Combined malonic and methylmalonic aciduria (CMAMMA) [MIM:614265]: A metabolic disease characterized by malonic and methylmalonic aciduria, with urinary excretion of much larger amounts of methylmalonic acid than malonic acid, in the presence of normal malonyl-CoA decarboxylase activity. Clinical features include coma, ketoacidosis, hypoglycemia, failure to thrive, microcephaly, dystonia, axial hypotonia and/or developmental delay, and neurologic manifestations including seizures, psychiatric disease and/or cognitive decline.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8

Sequence similarities: Belongs to the ATP-dependent AMP-binding enzyme family.

Sequence caution: The sequence AAH72391.1 differs from that shown. Reason: Aberrant splicing.