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Mouse Anti-HADHA Recombinant Antibody (CF192) (CBMAB-FT286LY)

The product is antibody recognizes HADHA . The antibody CF192 immunoassay techniques such as: ELISA, WB, IHC, IF.
See all HADHA antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
CF192
Antibody Isotype
IgG2a
Application
ELISA, WB, IHC, IF

Basic Information

Immunogen
hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein) , alpha subunit
Specificity
Human, Mouse
Antibody Isotype
IgG2a
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
50% glycerol
Preservative
0.02% sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha
Introduction
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]
Entrez Gene ID
Human3030
Mouse97212
UniProt ID
HumanP40939
MouseQ8BMS1
Alternative Names
HADH
Function
Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536).

The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090).

Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536).

Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536).

Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787).

It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787).

Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787).
Biological Process
Cardiolipin acyl-chain remodeling Source: UniProtKB
Fatty acid beta-oxidation Source: ComplexPortal
Response to insulin Source: Ensembl
Response to xenobiotic stimulus Source: Ensembl
Cellular Location
Mitochondrion; Mitochondrion inner membrane. Protein stability and association with mitochondrion inner membrane do not require HADHB.
Involvement in disease
Mitochondrial trifunctional protein deficiency (MTPD):
A disease biochemically characterized by loss of all enzyme activities of the mitochondrial trifunctional protein complex. Variable clinical manifestations include hypoglycemia, cardiomyopathy, delayed psychomotor development, sensorimotor axonopathy, generalized weakness, hepatic dysfunction, respiratory failure. Sudden infant death may occur. Most patients die from heart failure.
Long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency):
The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.
Maternal acute fatty liver of pregnancy (AFLP):
Severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).

Ding, J., Wu, L., Zhu, G., Zhu, J., Luo, P., & Li, Y. (2023). HADHA alleviates hepatic steatosis and oxidative stress in NAFLD via inactivation of the MKK3/MAPK pathway. Molecular Biology Reports, 50(2), 961-970.

Ma, M., Zhang, C., Cao, R., Tang, D., Sang, X., Zou, S., ... & Fu, X. (2022). UBE2O promotes lipid metabolic reprogramming and liver cancer progression by mediating HADHA ubiquitination. Oncogene, 41(48), 5199-5213.

Pan, A., Sun, X. M., Huang, F. Q., Liu, J. F., Cai, Y. Y., Wu, X., ... & Qi, L. W. (2022). The mitochondrial β-oxidation enzyme HADHA restrains hepatic glucagon response by promoting β-hydroxybutyrate production. Nature Communications, 13(1), 386.

Di Carlo, C., Sousa, B. C., Manfredi, M., Brandi, J., Dalla Pozza, E., Marengo, E., ... & Cecconi, D. (2021). Integrated lipidomics and proteomics reveal cardiolipin alterations, upregulation of HADHA and long chain fatty acids in pancreatic cancer stem cells. Scientific Reports, 11(1), 13297.

Liu, Y., Lu, L. L., Wen, D., Liu, D. L., Dong, L. L., Gao, D. M., ... & Wu, W. Z. (2020). MiR-612 regulates invadopodia of hepatocellular carcinoma by HADHA-mediated lipid reprogramming. Journal of hematology & oncology, 13(1), 1-19.

Yamamoto, K., Abe, S., Honda, A., Hashimoto, J., Aizawa, Y., Ishibashi, S., ... & Kitagawa, M. (2020). Fatty acid beta oxidation enzyme HADHA is a novel potential therapeutic target in malignant lymphoma. Laboratory Investigation, 100(3), 353-362.

Miklas, J. W., Clark, E., Levy, S., Detraux, D., Leonard, A., Beussman, K., ... & Ruohola-Baker, H. (2019). TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes. Nature communications, 10(1), 4671.

Liu, S., Liu, X., Wu, F., Zhang, X., Zhang, H., Gao, D., ... & Zhao, Z. (2019). HADHA overexpression disrupts lipid metabolism and inhibits tumor growth in clear cell renal cell carcinoma. Experimental Cell Research, 384(1), 111558.

Diebold, I., Schön, U., Horvath, R., Schwartz, O., Holinski-Feder, E., Kölbel, H., & Abicht, A. (2019). HADHA and HADHB gene associated phenotypes-Identification of rare variants in a patient cohort by Next Generation Sequencing. Molecular and cellular probes, 44, 14-20.

Thapa, D., Wu, K., Stoner, M. W., Xie, B., Zhang, M., Manning, J. R., ... & Scott, I. (2018). The protein acetylase GCN5L1 modulates hepatic fatty acid oxidation activity via acetylation of the mitochondrial β-oxidation enzyme HADHA. Journal of Biological Chemistry, 293(46), 17676-17684.

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For research use only. Not intended for any clinical use.

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