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anti-Human ACAA2 Antibodies:
anti-Rat (Rattus) ACAA2 Antibodies:
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Cow (Bovine) Polyclonal ACAA2 Primary Antibody for WB - ABIN2783320
Aboulaich, Vainonen, Strålfors, Vener: Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes. in The Biochemical journal 2004
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Human Monoclonal ACAA2 Primary Antibody for IF, IHC (p) - ABIN564548
Kuhlow, Zarse, Voigt, Schulz, Petzke, Schomburg, Pfeiffer, Ristow: Opposing effects of dietary sugar and saturated fat on cardiovascular risk factors and glucose metabolism in mitochondrially impaired mice. in European journal of nutrition 2010
Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA (show HADHA Antibodies) and HADHB (show HADHB Antibodies) mRNAs.
nonstructural protein 5 (show CAPS Antibodies) (NS5 (show RAF1 Antibodies)) interacted with hydroxyacyl-CoA dehydrogenase (show HADH Antibodies) alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP) involved in LCFA beta-oxidation
Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy.
Heterozygous mutation in HADHB (show HADHB Antibodies) gene cause early-onset axonal axonal Charcot-Marie-tooth disease.
The results demonstrated that ERbeta (show ESR2 Antibodies) was indeed associated and colocalized with HADHB (show HADHB Antibodies) within mitochondria.
HADHB (show HADHB Antibodies) is a functional molecular target of estrogen receptor alpha (show ESR1 Antibodies) in the mitochondria, and the interaction may play an important role in the estrogen-mediated lipid metabolism in animals and humans.
mutational analysis of the HADHB (show HADHB Antibodies) gene, which encodes long-chain 3-ketoacyl-CoA thiolase (show HADHB Antibodies), identified compound heterozygous mutations of c.520C>T (p.R141C) and c.1331G>A (p.R411K) in a case of mitochondrial trifunctional protein deficiency
Results emphasize the value of cDNA analysis in the characterization of HADHA (show HADHA Antibodies) and HADHB (show HADHB Antibodies) mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP defects.
Recombinant mitochondrial trifunctional protein displayed 2-enoyl-CoA hydratase, l-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase (show HADHB Antibodies) activities.
The present findings showed that all missense mutations in HADHB (show HADHB Antibodies) were disease-causing.
Data show that the major mitochondrial partner of Shc (show SHC1 Antibodies) adaptor protein p46Shc is the lipid oxidation enzyme 3-ketoacylCoA thiolase (show HADHB Antibodies) ACAA2, to which p46Shc binds directly and with a strong affinity.
This gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Alternatively spliced transcript variants have been found\; however, their full-length nature is not known.
3-ketoacyl-CoA thiolase, mitochondrial
, acetyl-Coenzyme A acyltransferase 2
, beta ketothiolase
, mitochondrial 3-oxoacyl-CoA thiolase
, mitochondrial 3-oxoacyl-Coenzyme A thiolase
, 2-enoyl-Coenzyme A (CoA) hydratase, beta subunit
, 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit
, acetyl-CoA acyltransferase
, hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit
, trifunctional enzyme subunit beta, mitochondrial
, acetyl-Coenzyme A acyltransferase 2 (mitochondrial 3-oxoacyl-Coenzyme A thiolase)
, mitochondrial acetyl-Coenzyme A acyltransferase 2