This gene encodes the beta subunit of the mitochondrial trifunctional protein, a catalyst of mitochondrial beta-oxidation of ... 2004). "General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations ... The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the ... "Trifunctional enzyme subunit beta, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Archived from the ...

The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the ... This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of ... The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit ... trifunctional protein), alpha subunit is a protein that in humans is encoded by the HADHA gene. Mutations in HADHA have been ...

MTP is a hetero-octamer composed of four alpha and four beta subunits: HADHA HADHB The three functions are 2-enoyl coenzyme A ( ... Mitochondrial trifunctional protein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out ... mitochondrial+trifunctional+protein+TP at the U.S. National Library of Medicine Medical Subject Headings (MeSH) (Proteins). ... Zhou, Z.; Zhou, J.; Du, Y. (2012). "Estrogen Receptor Alpha Interacts with Mitochondrial Protein HADHB and Affects Beta- ...

... mitochondrial ATP5PO: encoding enzyme subunit ATP synthase subunit O, mitochondrial B3GALT5: encoding enzyme beta-1,3- ... encoding GA-binding protein alpha chain GART: encoding enzyme trifunctional purine biosynthetic protein adenosine-3 GATD3A: ... encoding interleukin-10 receptor subunit beta ITGB2: encoding protein integrin beta-2 ITSN1: encoding protein intersectin-1 ... encoding protein encoding protein encoding protein Trafficking protein particle complex subunit 10 TMPRSS2: encoding enzyme ...

MLCL AT-1 is completely identical to the 59-kDa C-terminal end of mitochondrial trifunctional protein HADHA, suggesting the ... "Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidation". PLOS ONE. 7 (11): e48628. doi:10.1371/ ... MLCL AT-1 is likely a splice variant of the alpha subunit of MTP. Treatment with thyroxine, a thyroid hormone, can produce an ... and by comparing this protein against a human protein database, they identified a sequenced but uncharacterized human protein ...

002973 Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial ... subunit, beta type, 2 (PSMB2), mRNA PSMB3 NM_002795 PSMB4 NM_002796 Homo sapiens proteasome (prosome, macropain) subunit, beta ... 003001 HADHA Trifunctional protein subunit alpha COMT Catechol-O-methyl transferase) NDUFA2NM_002488 NDUFA3 NM_004542 NDUFA4 NM ... Protein translocation in ER SSR3 Translocon-associated protein TRAPG. Protein translocation in ER SUMO1 Protein targeting SUMO3 ...

The mechanism of this condition indicates that the mitochondrial trifunction protein catalyzes 3 steps in mitochondrial beta- ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ... Trifunctional protein deficiency is characterized by decreased activity of all 3 enzymes. Clinically, trifunctional protein ... trifunctional protein deficiency at Wikimedia Commons Scholia has a topic profile for Mitochondrial trifunctional protein ...

... trifunctional protein), beta subunit HSPC159: Galectin-related protein ID2-AS1: encoding protein Id2 antisense rna 1 (head to ... Protein arginine methyltransferase NDUFAF7, mitochondrial NRBP1: Nuclear receptor-binding protein 1 ODC1: Ornithine ... encoding protein LisH domain-containing protein ARMC9 B3GNT7: encoding protein UDP-GlcNAc:betaGal beta-1,3-N- ... encoding protein ZEB2-AS1 ZNF142: zinc finger protein 142 ZNF2: encoding protein Zinc finger protein 2 The following diseases ...

Also, it has been found to be a subunit of the peroxisomal trifunctional enzyme (pTFE) and contributes only to minor cleavages ... Janssen U, Stoffel W (May 2002). "Disruption of mitochondrial beta -oxidation of unsaturated fatty acids in the 3,2-trans-enoyl ... According to protein profiling in the human liver biopsies of HCV patients, a correlation was initially discovered between ... In enoyl-CoA isomerase, the two beta-sheets are part of the catalytic site, since the NH groups of residues following the beta- ...

... trifunctional protein), alpha subunit GRCh38: Ensembl release 89: ENSG00000138796 - Ensembl, May 2017 GRCm38: Ensembl release ... functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta- ... "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). Molven A, Matre ... The HADH gene encodes a 34.3 kDa protein that has 314 amino acids and 124 observed peptides. This gene is a member of the 3- ...