Acyl-CoA synthetase family member 3; mitochondrial; 621-; ACSF3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This ACSF3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 413-442 amino acids from the Central region of human ACSF3.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ACSF3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Cardiovascular; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

ACSF3 Antibody (Center) western blot analysis in mouse heart tissue lysates (15ug/lane).This demonstrates the ACSF3 antibody detected ACSF3 protein (arrow).

ACSF3 antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human tonsils tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the ACSF3 antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

64130

acyl-CoA synthetase family member 3

acyl-CoA synthetase family member 3, mitochondrial

Acyl-CoA synthetase family member 3, mitochondrial

ACSF3_HUMAN

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]

ACSF3: 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. Defects in ACSF3 are the cause of combined malonic and methylmalonic aciduria (CMAMMA). 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. Belongs to the ATP-dependent AMP-binding enzyme family.

Protein type: Ligase; EC 6.2.1.-; EC 6.-.-.-

Chromosomal Location of Human Ortholog: 16q24.3

Cellular Component: mitochondrion

Molecular Function: acid-thiol ligase activity; ATP binding

Biological Process: fatty acid metabolic process; fatty acid biosynthetic process

Disease: Combined Malonic And Methylmalonic Aciduria

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