HADHA

Official Full Name

hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha

Organism

Homo sapiens

Gene ID

3030

Background

This gene encodes the alpha 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 alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. 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. [provided by RefSeq, Jul 2008]

Synonyms

GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA

Cat.No.	Product Name	Price
CSC-DC006125	Panoply™ Human GATA6 Knockdown Stable Cell Line	Inquiry
CSC-DC006823	Panoply™ Human HADHA Knockdown Stable Cell Line	Inquiry
CSC-SC006125	Panoply™ Human GATA6 Over-expressing Stable Cell Line	Inquiry
CSC-SC006823	Panoply™ Human HADHA Over-expressing Stable Cell Line	Inquiry
CLKO-0903	HADHA KO Cell Lysate-HEK293T	Inquiry

Cat.No.	Product Name	Price
AD06628Z	Human GATA6 adenoviral particles	Inquiry
AD07315Z	Human HADHA adenoviral particles	Inquiry
LV13401L	human GATA6 (NM_005257) lentivirus particles	Inquiry
LV14372L	human HADHA (NM_000182) lentivirus particles	Inquiry

Cat.No.	Product Name	Price
SHH299590	shRNA set against Rat GATA6 (NM_019185.1)	Inquiry
SHH310157	shRNA set against Rat HADHA (NM_130826.2)	Inquiry
SHH078329	shRNA set against Mouse Gata6(NM_010258.3)	Inquiry
SHH078365	shRNA set against Rat Gata6(NM_019185.1)	Inquiry
SHH113213	shRNA set against Human HADHA(NM_000182.4)	Inquiry
SHH113231	shRNA set against Mouse Hadha(NM_178878.2)	Inquiry
SHH113249	shRNA set against Rat Hadha(NM_130826.2)	Inquiry
SHH299582	shRNA set against Human GATA6 (NM_005257.4)	Inquiry
SHH299586	shRNA set against Mouse GATA6 (NM_010258.3)	Inquiry
SHH310149	shRNA set against Human HADHA (NM_000182.4)	Inquiry
SHH310153	shRNA set against Mouse HADHA (NM_178878.2)	Inquiry
SHW005556	shRNA set against Chicken HADHA (NM_205056)	Inquiry
SHW005914	shRNA set against Chicken GATA6 (NM_205420)	Inquiry
SHW011889	shRNA set against Danio rerio HADHAA (NM_001105276)	Inquiry
SHW014987	shRNA set against Danio rerio GATA6 (NM_131557)	Inquiry

Cat.No.	Product Name	Price
CDCB158510	Human HADHA ORF clone (BC009235)	Inquiry
MiUTR3H-03715	HADHA miRNA 3'UTR clone	Inquiry
MiUTR1R-02371	HADHA miRNA 3'UTR clone	Inquiry
MiUTR1R-02055	GATA6 miRNA 3'UTR clone	Inquiry
MiUTR1M-05531	HADHA miRNA 3'UTR clone	Inquiry
MiUTR1M-04946	GATA6 miRNA 3'UTR clone	Inquiry
CDFR013942	Rat Hadha cDNA Clone(NM_130826.2)	Inquiry
CDFR011483	Rat Gata6 cDNA Clone(NM_019185.1)	Inquiry
CDFL004912	Mouse Gata6 cDNA Clone(NM_010258.3)	Inquiry
CDFH008273	Human HADHA cDNA Clone(NM_000182.4)	Inquiry
MiUTR3H-04294	GATA6 miRNA 3'UTR clone	Inquiry
SKO0514	HADHA Validated sgRNA vector	Inquiry
CDCG010508	Mouse HADHA ORF clone(NM_178878.2)	Inquiry
CDCB167389	Chicken GATA6 ORF Clone (NM_205420)	Inquiry
CDCR279121	Human HADHA ORF Clone(NM_000182.4)	Inquiry
CDCS405482	Human HADHA ORF Clone (BC009235)	Inquiry
CDCL110769	Human Gata6 ORF clone (NM_010258.3)	Inquiry
CDCG004990	Human GATA6 ORF clone(NM_005257.4)	Inquiry
CDCB191442	Rabbit GATA6 ORF clone (XM_008261133.1)	Inquiry
CDCB184923	Rabbit HADHA ORF clone (XM_002709814.2)	Inquiry
CDCB176462	Danio rerio GATA6 ORF Clone (NM_131557)	Inquiry
CDCB173364	Danio rerio HADHAA ORF Clone (NM_001105276)	Inquiry
CDCB167031	Chicken HADHA ORF Clone (NM_205056)	Inquiry
CDCR378356	Rat Gata6 ORF Clone(NM_019185.1)	Inquiry
CDCR380976	Rat Hadha ORF Clone(NM_130826.2)	Inquiry

Detailed Information

Recent Research Progress

The HADHA gene encodes the alpha subunit of the mitochondrial trifunctional protein (MTP), which is a lipid metabolism enzymes and thought to function in the mitochondria. However, HADHA was originally characterized as a gastrin-binding protein.

HADHA associates with human RNA silencing

HADHA is a new component in the human RNA silencing machinery. According to related research, HADHA colocalizes with dicer and is part of dicing complex. Further, overexpression of HADHA resulted in a higher abundance level of mature miRNA against a reduction in respective precursor level in HADHA knocked down cells, and vice versa. These findings suggest an auxiliary role for HADHA in miRNA biogenesis.

Thus some researches identify HADHA as a new dicer associated protein in HEK293T cells. In addition, functional studies revealed that, HADHA is involved in the processing of precursor into mature miRNA.

HADHA involved in long-chain fatty acid-induced autophagy in IECs

HADHA is potentially important for autophagy. Some researches proved that HADHA was expressed in not only the mitochondria but also the cytosol. Moreover, some data illustrate that HADHA in intestinal epithelial cells (IECs) as a novel LC3-binding molecule, and HADHA’s induction has association with LC3 puncta which is autophagy-associated protein. Furthermore, some researches demonstrated that HADHA is involved in long-chain fatty acid-induced (palmitic acid-induced) autophagy in IECs, which might support cell survival in IECs. Further investigations are required to clarify the mechanisms by which HADHA acts on LC3 formation. HADHA may have novel functions in autophagy and cell death signaling, so it may be a good target of immune regulation in the intestine.

Prognostic significance of HADHA in ccRCC

Related research shows HADHA was downregulated in clear cell renal cell carcinoma (ccRCC); further IHC analysis revealed that HADHA expression was significantly associated with tumor grade, stage, size, metastasis, and cancer-specific survival.Moreover, Bioinformatics analysis indicated HADHA and acetyl-coenzyme Aacetyltransferase 2 (ACAT2) interacted tightly and played vital roles in lipid metabolism. Collectively, these findings indicated that HADHA could serve as a promising prognostic marker in ccRCC, which indicated lipid metabolism abnormality might be involved in ccRCC tumorigenesis. Thus, expression of HADHA can be used as potential prognostic factors or therapeutic targets in patients with renal cell carcinoma(RCC).

Effect of HADHA in hepatic steatosis and cell injury in NAFLD

According to some researches, HADHA knockdown in L02 cells resulted in an increased of lipid accumulation and downregulation of gene expression involved in fatty acid beta-oxidation showing reduced levels of PPARα, ACOX1, CPT2, EHHADH, ECHS1, HADHB and HADH. Additionally, administering HADHA siRNA exhibited improvement of oxidative stress. Furthermore, HADHA knockdown in L02 cells demonstrated weakened of energy metabolism signaling pathway that is AMPK, activation of inflammatory- related signaling pathway MAPK and MKK3 pathway, and improve ER stress by downregulation of C/EBPα and C/EBPβ. Moreover, HADHA was regulated directly by upstream gene of miR-124, and silencing miR-124 led to an initial increase in mRNA and protein levels of HADHA. Thus, this research reported that HADHA upregulation in FFA induced nonalcoholic fatty liver disease(NAFLD) L02 cells, may play a protective role for steatosis via the upregulation of gene expression involved in fatty acid beta-oxidation and activation of AMPK pathway. These findings suggest that HADHA plays a role in hepatic steatosis and cell injury in NAFLD and provides a new insight into the pathogenic mechanisms of NAFLD, may becoming a potential new therapeutic target for NAFLD.

References:

Kakumani P K, et al. Association of HADHA with human RNA silencing machinery. Biochemical & Biophysical Research Communications, 2015, 466(3):481-485.
Maeyashiki C, et al. HADHA, the alpha subunit of the mitochondrial trifunctional protein, is involved in long-chain fatty acid-induced autophagy in intestinal epithelial cells. Biochemical & Biophysical Research Communications, 2017, 484(3):636-641.
Zhao Z, et al. Prognostic significance of two lipid metabolism enzymes, HADHA and ACAT2, in clear cell renal cell carcinoma. Tumour Biology the Journal of the International Society for Oncodevelopmental Biology & Medicine, 2015, 37(6):8121-8130.

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