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Acetyl-Coenzyme A Acyltransferase 2 (Mitochondrial 3-Oxoacyl-Coenzyme A Thiolase) Proteins (ACAA2)

ACAA2 encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids.

list all proteins Gene Name GeneID UniProt
ACAA2 3032 P55084
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Catalog No. Origin Source Conjugate Quantity Supplier Delivery Price Details
Human Un-conjugated 5 μg Log in to see 6 Days
$168.54
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More Proteins for Acetyl-Coenzyme A Acyltransferase 2 (Mitochondrial 3-Oxoacyl-Coenzyme A Thiolase) (ACAA2) Interaction Partners

Human Acetyl-Coenzyme A Acyltransferase 2 (Mitochondrial 3-Oxoacyl-Coenzyme A Thiolase) (ACAA2) interaction partners

  1. Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA (show HADHA Proteins) and HADHB (show HADHB Proteins) mRNAs.

  2. 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

  3. Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy.

  4. Heterozygous mutation in HADHB (show HADHB Proteins) gene cause early-onset axonal axonal Charcot-Marie-tooth disease.

  5. The results demonstrated that ERbeta (show ESR2 Proteins) was indeed associated and colocalized with HADHB (show HADHB Proteins) within mitochondria.

  6. 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.

  7. 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

  8. 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.

  9. Recombinant mitochondrial trifunctional protein displayed 2-enoyl-CoA hydratase, l-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase (show HADHB Proteins) activities.

  10. The present findings showed that all missense mutations in HADHB (show HADHB Proteins) were disease-causing.

ACAA2 Protein Profile

Protein Summary

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.

Gene names and symbols associated with ACAA2

  • hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit (HADHB)
  • ECHB protein
  • MTPB protein
  • TP-BETA protein

Protein level used designations for ACAA2

2-enoyl-Coenzyme A (CoA) hydratase, beta subunit , 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit , acetyl-CoA acyltransferase , beta-ketothiolase , hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit , trifunctional enzyme subunit beta, mitochondrial

GENE ID SPECIES
3032 Homo sapiens
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