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Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA (show HADHA Proteins) and HADHB (show HADHB Proteins) mRNAs.
nonstructural protein 5 (show CAPS Proteins) (NS5 (show RAF1 Proteins)) interacted with hydroxyacyl-CoA dehydrogenase (show HADH Proteins) alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP (show MT1B Proteins)) 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 Proteins) gene cause early-onset axonal axonal Charcot-Marie-tooth disease.
The results demonstrated that ERbeta (show ESR2 Proteins) was indeed associated and colocalized with HADHB (show HADHB Proteins) within mitochondria.
HADHB (show HADHB Proteins) is a functional molecular target of estrogen receptor alpha (show ESR1 Proteins) 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 Proteins) gene, which encodes long-chain 3-ketoacyl-CoA thiolase (show HADHB Proteins), 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 Proteins) and HADHB (show HADHB Proteins) mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP (show MT1B Proteins) defects.
Recombinant mitochondrial trifunctional protein displayed 2-enoyl-CoA hydratase, l-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase (show HADHB Proteins) activities.
The present findings showed that all missense mutations in HADHB (show HADHB Proteins) were disease-causing.
Data show that the major mitochondrial partner of Shc (show SHC1 Proteins) adaptor protein p46Shc is the lipid oxidation enzyme 3-ketoacylCoA thiolase (show HADHB Proteins) 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