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Mtpalpha Mitochondrial trifunctional protein alpha subunit [ Drosophila melanogaster (fruit fly) ]

Gene ID: 34276, updated on 5-Jul-2025
Official Symbol
Mtpalphaprovided by FlyBase
Official Full Name
Mitochondrial trifunctional protein alpha subunitprovided by FlyBase
Primary source
FLYBASE:FBgn0028479
Locus tag
Dmel_CG4389
See related
AllianceGenome:FB:FBgn0028479
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Drosophila melanogaster
Lineage
Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
Also known as
BcDNA:GH12558; CG 4389; CG4389; Dmel\CG4389; Hadha; HADHA; LBP; Mtp alpha
Summary
Predicted to enable enoyl-CoA hydratase activity and long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity. Involved in determination of adult lifespan; fatty acid beta-oxidation; and response to starvation. Located in peroxisome. Is expressed in adult head; adult heart; embryonic/larval lymph gland; organism; and spermatozoon. Used to study lipid metabolism disorder and steatotic liver disease. Human ortholog(s) of this gene implicated in mitochondrial trifunctional protein deficiency 1 and steatotic liver disease. Orthologous to human HADHA (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha). [provided by Alliance of Genome Resources, Jul 2025]
Orthologs
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See Mtpalpha in Genome Data Viewer
Location:
30B12-30B12; 2-35 cM
Exon count:
5
Annotation release Status Assembly Chr Location
Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 2L NT_033779.5 (9573563..9576769, complement)
Release 5.57 previous assembly Release 5 (GCF_000001215.2) 2L NT_033779.4 (9573563..9576769, complement)

Chromosome 2L - NT_033779.5Genomic Context describing neighboring genes Neighboring gene uncharacterized protein Neighboring gene junctophilin Neighboring gene long non-coding RNA:CR44149 Neighboring gene brickwall Neighboring gene uncharacterized protein Neighboring gene long non-coding RNA:CR43621 Neighboring gene glial cells missing

Products Interactant Other Gene Complex Source Pubs Description

Gene Ontology Provided by FlyBase

Function Evidence Code Pubs
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IEA
Inferred from Electronic Annotation
more info
 
enables NAD+ binding IEA
Inferred from Electronic Annotation
more info
 
enables catalytic activity IEA
Inferred from Electronic Annotation
more info
 
enables enoyl-CoA hydratase activity IBA
Inferred from Biological aspect of Ancestor
more info
 
enables enoyl-CoA hydratase activity IEA
Inferred from Electronic Annotation
more info
 
enables enoyl-CoA hydratase activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IBA
Inferred from Biological aspect of Ancestor
more info
 
enables long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
 
enables oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor IEA
Inferred from Electronic Annotation
more info
 
Process Evidence Code Pubs
involved_in determination of adult lifespan IMP
Inferred from Mutant Phenotype
more info
PubMed 
involved_in fatty acid beta-oxidation IBA
Inferred from Biological aspect of Ancestor
more info
 
involved_in fatty acid beta-oxidation IEA
Inferred from Electronic Annotation
more info
 
involved_in fatty acid beta-oxidation IMP
Inferred from Mutant Phenotype
more info
PubMed 
involved_in fatty acid beta-oxidation ISS
Inferred from Sequence or Structural Similarity
more info
 
involved_in fatty acid metabolic process IEA
Inferred from Electronic Annotation
more info
 
involved_in response to starvation IMP
Inferred from Mutant Phenotype
more info
PubMed 
involved_in wound healing HMP PubMed 
Component Evidence Code Pubs
part_of mitochondrial fatty acid beta-oxidation multienzyme complex IBA
Inferred from Biological aspect of Ancestor
more info
 
part_of mitochondrial fatty acid beta-oxidation multienzyme complex IEA
Inferred from Electronic Annotation
more info
 
located_in mitochondrial inner membrane ISS
Inferred from Sequence or Structural Similarity
more info
 
located_in mitochondrion HDA PubMed 
located_in mitochondrion IEA
Inferred from Electronic Annotation
more info
 
located_in mitochondrion ISM
Inferred from Sequence Model
more info
PubMed 
located_in peroxisome IDA
Inferred from Direct Assay
more info
PubMed 
located_in peroxisome ISM
Inferred from Sequence Model
more info
PubMed 
located_in peroxisome ISS
Inferred from Sequence or Structural Similarity
more info
PubMed 
Preferred Names
mitochondrial trifunctional protein alpha subunit
Names
CG4389-PA
CG4389-PB
Mtpalpha-PA
Mtpalpha-PB
NP_609299.1
NP_723470.1

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

  1. NT_033779.5 Reference assembly

    Range
    9573563..9576769 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_164860.2NP_723470.1  mitochondrial trifunctional protein alpha subunit, isoform B

    See identical proteins and their annotated locations for NP_723470.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q8IPE8
    Related
    FBpp0079453
    Conserved Domains (4) summary
    cd06558
    Location:13196
    crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...
    TIGR02441
    Location:1736
    fa_ox_alpha_mit; fatty acid oxidation complex, alpha subunit, mitochondrial
    pfam00725
    Location:517612
    3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain
    pfam02737
    Location:336514
    3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain
  2. NM_135455.3NP_609299.1  mitochondrial trifunctional protein alpha subunit, isoform A

    See identical proteins and their annotated locations for NP_609299.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    Q9V397
    Related
    FBpp0079454
    Conserved Domains (4) summary
    cd06558
    Location:52235
    crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...
    TIGR02441
    Location:38775
    fa_ox_alpha_mit; fatty acid oxidation complex, alpha subunit, mitochondrial
    pfam00725
    Location:556651
    3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain
    pfam02737
    Location:375553
    3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain