ACAD9 (GeneName), Acyl-CoA dehydrogenase family member 9, mitochondrial (ProteinName), ACAD9_HUMAN.
Human ACAD9/ Acyl-CoA dehydrogenase family member 9, mitochondrial ELISA Kit
Natural and recombinant human Acyl-CoA dehydrogenase family member 9, mitochondrial
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Part of the mitochondrial complex I assembly (MCIA) complex. The complex comprises at least TMEM126B, NDUFAF1, ECSIT, and ACAD9 (By similarity). Interacts with NDUFAF1 and ECSIT (PubMed:20816094).
Required for mitochondrial complex I assembly (PubMed:20816094, PubMed:24158852). Has a dehydrogenase activity on palmitoyl-CoA (C16:0) and stearoyl-CoA (C18:0). It is three times more active on palmitoyl-CoA than on stearoyl-CoA. However, it does not play a primary role in long-chain fatty acid oxidation in vivo (PubMed:20816094, PubMed:24158852). Has little activity on octanoyl-CoA (C8:0), butyryl-CoA (C4:0) or isovaleryl-CoA (5:0).
MSGCGLFLRT | TAAARACRGL | VVSTANRRLL | RTSPPVRAFA | KELFLGKIKK |
KEVFPFPEVS | QDELNEINQF | LGPVEKFFTE | EVDSRKIDQE | GKIPDETLEK |
LKSLGLFGLQ | VPEEYGGLGF | SNTMYSRLGE | IISMDGSITV | TLAAHQAIGL |
KGIILAGTEE | QKAKYLPKLA | SGEHIAAFCL | TEPASGSDAA | SIRSRATLSE |
DKKHYILNGS | KVWITNGGLA | NIFTVFAKTE | VVDSDGSVKD | KITAFIVERD |
FGGVTNGKPE | DKLGIRGSNT | CEVHFENTKI | PVENILGEVG | DGFKVAMNIL |
NSGRFSMGSV | VAGLLKRLIE | MTAEYACTRK | QFNKRLSEFG | LIQEKFALMA |
QKAYVMESMT | YLTAGMLDQP | GFPDCSIEAA | MVKVFSSEAA | WQCVSEALQI |
LGGLGYTRDY | PYERILRDTR | ILLIFEGTNE | ILRMYIALTG | LQHAGRILTT |
RIHELKQAKV | STVMDTVGRR | LRDSLGRTVD | LGLTGNHGVV | HPSLADSANK |
FEENTYCFGR | TVETLLLRFG | KTIMEEQLVL | KRVANILINL | YGMTAVLSRA |
SRSIRIGLRN | HDHEVLLANT | FCVEAYLQNL | FSLSQLDKYA | PENLDEQIKK |
VSQQILEKRA | YICAHPLDRT | C
This product has not yet been referenced specifically in any publications.
"Cloning and functional characterization of ACAD-9, a novel member of human acyl-CoA dehydrogenase family."
"Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I."
"Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency."
"A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency."
"ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies."
"Riboflavin-responsive oxidative phosphorylation complex I deficiency caused by defective ACAD9: new function for an old gene."
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."
"Carnitine palmitoyltransferase I and Acyl-CoA dehydrogenase 9 in retina: insights of retinopathy in mitochondrial trifunctional protein defects."
"Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain."
"Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids."
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