製品: HADHA Recombinant Rabbit mAb
カタログ: BF3919
タンパク質の説明: Rabbit monoclonal antibody to HADHA
アプリケーション: WB IHC IF/ICC
反応性: Human, Mouse, Rat
分子量: 78 kDa(Observed); 83kD(Calculated).
ユニプロット: P40939

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製品説明

ソース:
Rabbit IgG
アプリケーション:
WB 1:1000, IHC 1:100, ICC
*The optimal dilutions should be determined by the end user. For optimal experimental results, antibody reuse is not recommended.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

反応性:
Human, Mouse, Rat
クローナリティ:
Monoclonal [ReFirm23366]
特異性:
HADHA Recombinant Rabbit mAb detects endogenous levels of HADHA.
コンジュゲート:
Unconjugated.
精製:
Affinity-chromatography.
保存:
Rabbit IgG in Tris-Glycine (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
別名:

折りたたみ/展開

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

免疫原

免疫原:

A synthetic peptide from human HADHA

Uniprot:
遺伝子(ID):
タンパク質の説明:
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.
タンパク質配列:
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ

研究背景

機能:

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.

細胞の位置付け:

Mitochondrion. Mitochondrion inner membrane.
Note: Protein stability and association with mitochondrion inner membrane do not require HADHB.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
タンパク質ファミリー:

In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.

In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.

研究領域

· Metabolism > Lipid metabolism > Fatty acid elongation.

· Metabolism > Lipid metabolism > Fatty acid degradation.

· Metabolism > Amino acid metabolism > Valine, leucine and isoleucine degradation.

· Metabolism > Amino acid metabolism > Lysine degradation.

· Metabolism > Amino acid metabolism > Tryptophan metabolism.

· Metabolism > Metabolism of other amino acids > beta-Alanine metabolism.

· Metabolism > Carbohydrate metabolism > Propanoate metabolism.

· Metabolism > Carbohydrate metabolism > Butanoate metabolism.

· Metabolism > Lipid metabolism > Biosynthesis of unsaturated fatty acids.

· Metabolism > Global and overview maps > Metabolic pathways.

· Metabolism > Global and overview maps > Carbon metabolism.

· Metabolism > Global and overview maps > Fatty acid metabolism.

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