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HADHB

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GENERAL INFORMATION

Gene name

 HADHB

Gene description

 Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit

Protein class

 Disease related genes
Enzymes
Potential drug targets
Predicted intracellular proteins
Predicted secreted proteins

Predicted localization

 Intracellular,Secreted

Number of transcripts

 7
SHOW MORE

HUMAN PROTEIN ATLAS INFORMATION

Summary

 Localized to the mitochondria.

RNA cell category

 Expressed in all

Protein evidence

 Evidence at protein level

Main location

 Localized to the Mitochondria (supported)

Single-cell variation

 Single-cell variation in protein expression observed.

DATA RELIABILITY

Reliability score

 Supported based on 1 antibody.
HPA066099
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ASSAYS
RNA expression
Human cells

RNA EXPRESSION OVERVIEW
RNA cell line category: Expressed in all
Organ
Origin
Category
Expression
Alphabetical
Cell lines sorted after organ of phenotypic resemblance.

HUMAN CELLS

Summary

 Localized to the mitochondria.

Main location

 Mitochondria (supported)

 

 

 
Toggle channels
Low
High
G1
S
G2
M
N/A

Thumbnail

Antibody

Cell line

Location

Single-cell variation

Cell cycle dependent variation
HPA066099 Hep G2 Mitochondria
HPA066099 SK-MEL-30 Mitochondria Intensity
HPA066099 U-2 OS Mitochondria

GENE INFORMATION

Gene name

 HADHB (HGNC Symbol)

Synonyms

 MTPB

Description

 Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit (HGNC Symbol)

Entrez gene 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. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013]

Chromosome

 2

Cytoband

 p23.3

Chromosome location (bp)

 26243170 - 26290468

Protein evidence

 Evidence at protein level (all genes)

Ensembl

 ENSG00000138029 (version 83.38)

Entrez gene

 3032

UniProt

 P55084 (UniProt - Evidence at protein level)

neXtProt

 NX_P55084

Antibodypedia

 HADHB antibodies

PROTEIN BROWSER
HADHB-001
HADHB-002
HADHB-004
HADHB-005
HADHB-007
HADHB-201
HADHB-202

PROTEIN INFORMATION

Splice variant

UniProt

Protein class

Gene ontology

Length & mass

Signal peptide
(predicted)

Transmembrane regions
(predicted)
HADHB-001
ENSP00000325136
ENST00000317799
P55084 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase
Show all
Enzymes
   ENZYME proteins
   Transferases
   SPOCTOPUS predicted membrane proteins
Predicted intracellular proteins
Disease related genes
Potential drug targets
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0003857 [3-hydroxyacyl-CoA dehydrogenase activity]
GO:0003988 [acetyl-CoA C-acyltransferase activity]
GO:0004300 [enoyl-CoA hydratase activity]
GO:0005515 [protein binding]
GO:0005739 [mitochondrion]
GO:0005740 [mitochondrial envelope]
GO:0005741 [mitochondrial outer membrane]
GO:0005743 [mitochondrial inner membrane]
GO:0005783 [endoplasmic reticulum]
GO:0006635 [fatty acid beta-oxidation]
GO:0006644 [phospholipid metabolic process]
GO:0008152 [metabolic process]
GO:0016509 [long-chain-3-hydroxyacyl-CoA dehydrogenase activity]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
GO:0035965 [cardiolipin acyl-chain remodeling]
GO:0042645 [mitochondrial nucleoid]
GO:0044255 [cellular lipid metabolic process]
GO:0044281 [small molecule metabolic process]
GO:0044822 [poly(A) RNA binding]
GO:0046474 [glycerophospholipid biosynthetic process]
GO:0070062 [extracellular exosome]
Show all
 474 aa
51.3 kDa 		No 0
HADHB-002
ENSP00000385411
ENST00000405867
B5MD38 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial
Show all
   SPOCTOPUS predicted membrane proteins
   SPOCTOPUS predicted secreted proteins
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
Show all
 351 aa
37.9 kDa 		No 0
HADHB-004
ENSP00000404633
ENST00000425035
C9JE81 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
Show all
 176 aa
18.8 kDa 		No 0
HADHB-005
ENSP00000413103
ENST00000412805
C9K0M0 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial
Show all
   SPOCTOPUS predicted secreted proteins
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
Show all
 85 aa
9.4 kDa 		No 0
HADHB-007
ENSP00000415300
ENST00000448743
C9JEY0 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
Show all
 150 aa
16.1 kDa 		No 0
HADHB-201
ENSP00000444295
ENST00000537713
F5GZQ3 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial
Show all
   SPOCTOPUS predicted membrane proteins
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
Show all
 459 aa
49.6 kDa 		No 0
HADHB-202
ENSP00000442665
ENST00000545822
P55084 [Direct mapping]
Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase
Show all
Enzymes
   ENZYME proteins
   Transferases
   SPOCTOPUS predicted membrane proteins
Predicted secreted proteins
   Secreted proteins predicted by MDSEC
   SignalP predicted secreted proteins
   Phobius predicted secreted proteins
Disease related genes
Potential drug targets
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824 [catalytic activity]
GO:0003857 [3-hydroxyacyl-CoA dehydrogenase activity]
GO:0003988 [acetyl-CoA C-acyltransferase activity]
GO:0004300 [enoyl-CoA hydratase activity]
GO:0005515 [protein binding]
GO:0005739 [mitochondrion]
GO:0005740 [mitochondrial envelope]
GO:0005741 [mitochondrial outer membrane]
GO:0005743 [mitochondrial inner membrane]
GO:0005783 [endoplasmic reticulum]
GO:0006635 [fatty acid beta-oxidation]
GO:0006644 [phospholipid metabolic process]
GO:0008152 [metabolic process]
GO:0016747 [transferase activity, transferring acyl groups other than amino-acyl groups]
GO:0035965 [cardiolipin acyl-chain remodeling]
GO:0042645 [mitochondrial nucleoid]
GO:0044255 [cellular lipid metabolic process]
GO:0044281 [small molecule metabolic process]
GO:0044822 [poly(A) RNA binding]
GO:0046474 [glycerophospholipid biosynthetic process]
GO:0070062 [extracellular exosome]
Show all
 452 aa
48.9 kDa 		Yes 0

Contact

The Project

The Human Protein Atlas

The Human Protein Atlas project is funded
by the Knut & Alice Wallenberg foundation.