Hadh hydroxyacyl-Coenzyme A dehydrogenase [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Hadhprovided by MGI
Official Full Name: hydroxyacyl-Coenzyme A dehydrogenaseprovided by MGI
Primary source: MGI:MGI:96009
See related: Ensembl:ENSMUSG00000027984 AllianceGenome:MGI:96009
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: HCDH; Schad; Hadhsc
Summary: Enables 3-hydroxyacyl-CoA dehydrogenase activity. Involved in positive regulation of cold-induced thermogenesis and regulation of insulin secretion. Located in mitochondrion. Is expressed in several structures, including alimentary system; brain; genitourinary system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in familial hyperinsulinemic hypoglycemia 4. Orthologous to human HADH (hydroxyacyl-CoA dehydrogenase). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Broad expression in heart adult (RPKM 232.8), kidney adult (RPKM 182.9) and 21 other tissues See more
Orthologs: human all
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Genomic context

Location:: 3 G3; 3 60.86 cM

Exon count:: 8

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	3	NC_000069.7 (131027068..131065750, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	3	NC_000069.6 (131233419..131272101, complement)

Chromosome 3 - NC_000069.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Hadh deficiency induced oligoasthenoteratozoospermia through the TNF-alpha/Bcl-2 pathway in male mice.
Title: Hadh deficiency induced oligoasthenoteratozoospermia through the TNF-α/Bcl-2 pathway in male mice.
A physical association between short-chain 3-hydroxyacyl-coenzyme A dehydrogenase and important components of other key metabolic pathways. Most of the interactions were with enzymes in mitochondrial pathways.
Title: Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways.
SCHAD is involved in thermogenesis, in the maintenance of body weight, and in the regulation of nutrient-stimulated insulin secretion
Title: Role of medium- and short-chain L-3-hydroxyacyl-CoA dehydrogenase in the regulation of body weight and thermogenesis.
SCHAD deficiency causes hyperinsulinism by activation of GDH via loss of inhibitory regulation of GDH by SCHAD.
Title: Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase.
SCHAD regulates insulin secretion through a KATP channel-independent mechanism.
Title: Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents.
Results demonstrate that L-3-hydroxyacyl-CoA dehydrogenase type II (HADH II/ABAD) modulates 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity and suggests that mimetics may provide protective benefit in the treatment of Parkinson disease.
Title: L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinson's disease.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (4) 1 citation
Gene trapped (2)
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

Hadh (e-PCR), detects polymorphism
- UniSTS:527382

RH136485 (e-PCR)
- UniSTS:210469

AW742602 (e-PCR)
- UniSTS:207565

Hadh (e-PCR), detects polymorphism
- UniSTS:143312

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 3-hydroxyacyl-CoA dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info
enables 3-hydroxyacyl-CoA dehydrogenase activity	IDA Inferred from Direct Assay more info	PubMed
enables 3-hydroxyacyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables 3-hydroxyacyl-CoA dehydrogenase activity	ISS Inferred from Sequence or Structural Similarity more info
enables NAD+ binding	ISO Inferred from Sequence Orthology more info
enables NAD+ binding	ISS Inferred from Sequence or Structural Similarity more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables identical protein binding	ISS Inferred from Sequence or Structural Similarity more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed

Process	Evidence Code	Pubs
involved_in cell differentiation	IEA Inferred from Electronic Annotation more info
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	ISO Inferred from Sequence Orthology more info
involved_in fatty acid beta-oxidation	ISS Inferred from Sequence or Structural Similarity more info
involved_in negative regulation of insulin secretion	IEA Inferred from Electronic Annotation more info
involved_in negative regulation of insulin secretion	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of cold-induced thermogenesis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in regulation of insulin secretion	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in response to activity	IEA Inferred from Electronic Annotation more info
involved_in response to activity	ISO Inferred from Sequence Orthology more info
involved_in response to insulin	IEA Inferred from Electronic Annotation more info
involved_in response to xenobiotic stimulus	IEA Inferred from Electronic Annotation more info
involved_in response to xenobiotic stimulus	ISO Inferred from Sequence Orthology more info
involved_in spermatogenesis	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in cytoplasm	ISO Inferred from Sequence Orthology more info
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in mitochondrial inner membrane	HDA more info	PubMed
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleoplasm	IEA Inferred from Electronic Annotation more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

Names: L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; hydroxylacyl-Coenzyme A dehydrogenase-dehydrogenase; medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase; short-chain 3-hydroxyacyl-CoA dehydrogenase

NP_032238.2

EC 1.1.1.35

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_008212.4 → NP_032238.2 hydroxyacyl-coenzyme A dehydrogenase, mitochondrial precursor

See identical proteins and their annotated locations for NP_032238.2

Status: VALIDATED

Source sequence(s)

AC123634, AI845880, AK132260, AK148486

Consensus CDS

CCDS38640.1

UniProtKB/Swiss-Prot

Q3TF75, Q3THK8, Q3UFI0, Q61425, Q8K149, Q925U9

Related

ENSMUSP00000029610.9, ENSMUST00000029610.9

Conserved Domains (3) summary

COG1250
Location:27 → 313

FadB; 3-hydroxyacyl-CoA dehydrogenase [Lipid transport and metabolism]

pfam00725
Location:216 → 313

3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain

pfam02737
Location:29 → 214

3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain