HADHA Antibody - #DF7288

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製品: HADHA Antibody
カタログ: DF7288
タンパク質の説明: Rabbit polyclonal antibody to HADHA
アプリケーション: WB IHC IF/ICC
反応性: Human, Mouse, Rat
予測: Pig, Bovine, Horse, Sheep, Rabbit, Chicken, Xenopus
分子量: 83kDa; 83kD(Calculated).
ユニプロット: P40939
RRID: AB_2839227

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MS Report
HPLC Report
MSDS
説明書
COA
プロトコル
WB Handbook
IHC Protocol
IF/ICC Protocol

製品説明

ソース:
Rabbit
アプリケーション:
WB 1:500-1:2000, IHC 1:50-1:200, IF/ICC 1:100-1:500
*The optimal dilutions should be determined by the end user.
*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
予測:
Pig(100%), Bovine(100%), Horse(100%), Sheep(100%), Rabbit(100%), Chicken(100%), Xenopus(100%)
クローナリティ:
Polyclonal
特異性:
HADHA Antibody detects endogenous levels of total HADHA.
RRID:
AB_2839227
引用形式: Affinity Biosciences Cat# DF7288, RRID:AB_2839227.
コンジュゲート:
Unconjugated.
精製:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
保存:
Rabbit IgG in phosphate buffered saline , 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

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;

免疫原

免疫原:
Uniprot:

P40939(ECHA_HUMAN) >>Visit HPA database.

遺伝子(ID):
HADHA(3030)
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

種類予測

種類予測:

Score>80(red) has high confidence and is suggested to be used for WB detection. *The prediction model is mainly based on the alignment of immunogen sequences, the results are for reference only, not as the basis of quality assurance.

Species
Results
Score
Pig
100
Horse
100
Bovine
100
Sheep
100
Xenopus
100
Chicken
100
Rabbit
100
Dog
0
Zebrafish
0
Model Confidence:
High(score>80) Medium(80>score>50) Low(score<50) No confidence

PTMs - P40939 基板として

Site PTM Type Enzyme
K60 Acetylation
K60 Ubiquitination
T117 Phosphorylation
S120 Phosphorylation
K129 Acetylation
K129 Ubiquitination
K163 Acetylation
T167 Phosphorylation
T171 Phosphorylation
T203 Phosphorylation
S206 Phosphorylation
K214 Ubiquitination
Y239 Phosphorylation
K249 Acetylation
S257 Phosphorylation
K259 Acetylation
K285 Acetylation
K289 Acetylation
T294 Phosphorylation
K295 Acetylation
Y298 Phosphorylation
K303 Acetylation
K303 Ubiquitination
K309 Ubiquitination
S316 Phosphorylation
C322 S-Nitrosylation
K326 Acetylation
K334 Acetylation
K350 Acetylation
K350 Ubiquitination
K351 Acetylation
K353 Acetylation
K353 Ubiquitination
K359 Acetylation
K359 Ubiquitination
K383 Acetylation
K383 Ubiquitination
T387 Phosphorylation
K390 Acetylation
K390 Ubiquitination
T393 Phosphorylation
T395 Phosphorylation
R399 Methylation
K406 Acetylation
K406 Ubiquitination
K411 Acetylation
K413 Acetylation
K415 Ubiquitination
T418 Phosphorylation
S419 Phosphorylation
S427 Phosphorylation
Y435 Phosphorylation
S453 Phosphorylation
K460 Acetylation
K489 Ubiquitination
Y499 Phosphorylation
S501 Phosphorylation
K505 Acetylation
K519 Acetylation
K519 Ubiquitination
T521 Phosphorylation
S522 Phosphorylation
S524 Phosphorylation
K531 Ubiquitination
K540 Acetylation
Y546 Phosphorylation
R549 Methylation
K569 Acetylation
K570 Acetylation
K597 Acetylation
K605 Acetylation
K605 Ubiquitination
K625 Ubiquitination
Y637 Phosphorylation
Y639 Phosphorylation
K644 Acetylation
K646 Ubiquitination
S650 Phosphorylation
S665 Phosphorylation
S668 Phosphorylation
S669 Phosphorylation
Y724 Phosphorylation
K728 Acetylation
K728 Ubiquitination
K735 Ubiquitination
K742 Acetylation
K742 Ubiquitination
T745 Phosphorylation
S756 Phosphorylation
K759 Acetylation
K760 Acetylation

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
サブユニット構造:

Heterotetramer of 2 alpha/HADHA and 2 beta/HADHB subunits; forms the mitochondrial trifunctional enzyme. Also purified as higher order heterooligomers including a 4 alpha/HADHA and 4 beta/HADHB heterooligomer which physiological significance remains unclear.

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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