Product Details

Purity

>85% (SDS-PAGE)

Target Names

HADHA

Uniprot No.

P40939

Alternative Names

3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit; 3 oxoacyl CoA thiolase; 78 kDa gastrin binding protein; 78 kDa gastrin-binding protein; ECHA; ECHA_HUMAN; GBP; HADH; HADHA; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; LCEH; LCHAD; Long chain 3-hydroxyacyl-CoA dehydrogenase; Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit; Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit; Mitochondrial trifunctional enzyme alpha subunit; Mitochondrial trifunctional protein alpha subunit; MTPA; Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; TP ALPHA; TP-alpha; Trifunctional enzyme subunit alpha mitochondrial precursor

Species

Homo sapiens (Human)

Expression Region

37-763

Target Protein Sequence

TRTH INYGVKGDVA VVRINSPNSK VNTLSKELHS EFSEVMNEIW ASDQIRSAVL ISSKPGCFIA GADINMLAAC KTLQEVTQLS QEAQRIVEKL EKSTKPIVAA INGSCLGGGL EVAISCQYRI ATKDRKTVLG TPEVLLGALP GAGGTQRLPK MVGVPAALDM MLTGRSIRAD RAKKMGLVDQ LVEPLGPGLK PPEERTIEYL EEVAITFAKG LADKKISPKR DKGLVEKLTA YAMTIPFVRQ QVYKKVEEKV RKQTKGLYPA PLKIIDVVKT GIEQGSDAGY LCESQKFGEL VMTKESKALM GLYHGQVLCK KNKFGAPQKD VKHLAILGAG LMGAGIAQVS VDKGLKTILK DATLTALDRG QQQVFKGLND KVKKKALTSF ERDSIFSNLT GQLDYQGFEK ADMVIEAVFE DLSLKHRVLK EVEAVIPDHC IFASNTSALP ISEIAAVSKR PEKVIGMHYF SPVDKMQLLE IITTEKTSKD TSASAVAVGL KQGKVIIVVK DGPGFYTTRC LAPMMSEVIR ILQEGVDPKK LDSLTTSFGF PVGAATLVDE VGVDVAKHVA EDLGKVFGER FGGGNPELLT QMVSKGFLGR KSGKGFYIYQ EGVKRKDLNS DMDSILASLK LPPKSEVSSD EDIQFRLVTR FVNEAVMCLQ EGILATPAEG DIGAVFGLGF PPCLGGPFRF VDLYGAQKIV DRLKKYEAAY GKQFTPCQLL ADHANSPNKK FYQ

Protein Length

Full Length of Mature Protein

Tag Info

Tag type will be determined during the manufacturing process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.

Form

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Buffer before Lyophilization

Tris/PBS-based buffer, 6% Trehalose, pH 8.0

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet

Please contact us to get it.

Target Background

Function

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. 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. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. 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. Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity. 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. Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity.

Gene References into Functions

study indicate described high frequency of c.1528G>C variant of HADHA gene in Kashubian population, suggesting the founder effect. For the first time we have found high frequency of rs71441018 in the South Poland Silesian population PMID: 29095929
Low HADHA expression is associated with clear cell renal cell carcinoma. PMID: 26715271
Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA and HADHB mRNAs. PMID: 26109258
findings suggest an auxiliary role for HADHA in miRNA biogenesis and help in better understanding of molecular mechanisms underlying RNAi pathway PMID: 26367179
nonstructural protein 5 (NS5) interacted with hydroxyacyl-CoA dehydrogenase alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP) involved in LCFA beta-oxidation PMID: 25816318
revealed a novel G/A allelic change in the intronic region [chromosomal position 26417674; 20 bp adjacent to an already published SNP (rs151243950)] for one among the three acute fatty liver of pregnancy patients PMID: 24105666
Crystal structures of human mitochondrial 3-ketoacyl-CoA thiolase (hT1) in the apo form and in complex with CoA have been determined at 2.0 A resolution. The structures confirm the tetrameric quaternary structure of this degradative thiolase. PMID: 25478839
Mitochondrial Trifunctional-Protein depletion during hepatitis c virus infection rendered cells less responsive to alpha interferon treatment by impairing IFN-stimulated gene expression. PMID: 25673715
Report probable Kashubian origin of the prevalent HADHA c.1528G>C mutation responsible for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency in Poland. PMID: 20814823
Free fatty acids might affect the expression of mitochondrial beta-oxidation enzyme of LCHAD in trophoblast cells. PMID: 23253803
mitochondrial alphaTFP exhibits both in vitro and in vivo MLCL AT activity linking an enzyme of mitochondrial beta-oxidation to Cardiolipin remodeling. PMID: 23152787
these findings lend support to the hypothesis that mutations in the HADHA gene may be directly associated with and potentially causative of Maternal floor infarction/massive perivillous fibrin deposition PMID: 22746996
TP-alpha, collagen alpha-1(VI) chain and S100A9 are potential biomarkers of esophageal squamous cell carcinoma, and may play an important role in tumorigenesis and development of ESCC. PMID: 22583932
Both children were homozygous for the common mutation c.1528G>C and the parents were heterozygous for this same mutation. PMID: 22325456
High HADHA protein expression is associated with response to platinum-based chemotherapy for lung cancer. PMID: 22471497
Results emphasize the value of cDNA analysis in the characterization of HADHA and HADHB mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP defects. PMID: 21549624
Recombinant mitochondrial trifunctional protein displayed 2-enoyl-CoA hydratase, l-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase activities. PMID: 20825197
The G1528C (E474Q) mutation in the alpha-subunit of the mitochondrial trifunctional protein was not found in 10 women with acute fatty liver of pregnancy. PMID: 11978893
Plasma concentration varied more than erythrocyte levels. Postprandial period and fasting. PMID: 12971430
Both alpha- and beta-subunit mutations result in TFP complex instability, demonstrating that the mechanism of disease is the same in alpha- or beta-mutation-derived disease and explaining the biochemical and clinical similarities. PMID: 14630990
The retinal pigment epithelium rather than the choriocapillaris could be the critical affected cell layer in LCHAD retinopathy PMID: 15347768
DNA analysis of patients 1 and 2 revealed homozygosity for a c.1689+2T>G mutation of the HADHA gene, resulting in the skipping of exon 16 with an in-frame 69-bp deletion. PMID: 17143551
Neither maternal nor fetal heterozygosity for the E474Q mutation is a relevant factor of HELLP syndrome. PMID: 17313315
There might be diverse etiological factors in China contributing to AFLP other than the frequently reported mutation in the LCHAD. PMID: 18031367
The second of 220 SIDs cases was a compound heterozygous for the prevalent MTP G1528C mutation and a novel 1 bp deletion in exon 18 of the MTPalpha-subunit gene. PMID: 18045290
Severe cardiac mitochondrial proliferation and TFP deficiency was observed in intrauterine cardiomyopathy. PMID: 18485779
identify MLCL AT-1 as a human mitochondrial monolysocardiolipin acyltransferase involved in the remodeling of cardiolipin PMID: 19737925

Hide All

Involvement in disease

Mitochondrial trifunctional protein deficiency (MTPD); Long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency); Maternal acute fatty liver of pregnancy (AFLP)

Subcellular Location

Mitochondrion. Mitochondrion inner membrane.

Protein Families

Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family

Database Links

HGNC: 4801

OMIM: 600890

KEGG: hsa:3030

STRING: 9606.ENSP00000370023

UniGene: Hs.516032

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Recombinant Human Trifunctional enzyme subunit alpha, mitochondrial (HADHA)

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