产品: HADHA 抗体
货号: DF7288
描述: Rabbit polyclonal antibody to HADHA
应用: WB IHC IF/ICC
反应: Human, Mouse, Rat
预测: Pig, Bovine, Horse, Sheep, Rabbit, Chicken, Xenopus
分子量: 70-83kD; 83kD(Calculated).
蛋白号: P40939
RRID: AB_2839227

浏览相似产品>>

   规格 价格 库存
 50ul RMB¥ 1250 现货
 100ul RMB¥ 2300 现货
 200ul RMB¥ 3000 现货

货期: 当天发货

联系销售

产品描述

来源:
Rabbit
应用:
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: 适用于变性蛋白样本的免疫印迹检测. IHC: 适用于组织样本的石蜡(IHC-p)或冰冻(IHC-f)切片样本的免疫组化/荧光检测. IF/ICC: 适用于细胞样本的荧光检测. ELISA(peptide): 适用于抗原肽的ELISA检测.

反应:
Human, Mouse, Rat
预测:
Pig(100%), Bovine(%), Horse(%), Sheep(%), Rabbit(%), Chicken(%), Xenopus(%)
克隆:
Polyclonal
特异性:
HADHA Antibody detects endogenous levels of total HADHA.
RRID:
AB_2839227
引用格式: Affinity Biosciences Cat# DF7288, RRID:AB_2839227.
偶联:
Unconjugated. 130
纯化:
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.
别名:

展开/折叠

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;

抗原和靶标

免疫原:
Uniprot:
基因/基因ID:
描述:
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.
序列:
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ

种属预测

种属预测:

score>80的预测可信度较高,可尝试用于WB检测。*预测模型主要基于免疫原序列比对,结果仅作参考,不作为质保凭据。

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

研究背景

功能:

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.

细胞定位:

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
亚基结构:

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.

蛋白家族:

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.

研究领域

· 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.

限制条款

产品的规格、报价、验证数据请以官网为准,官网链接:www.affbiotech.com | www.affbiotech.cn(简体中文)| www.affbiotech.jp(日本語)

产品的数据信息为Affinity所有,未经授权不得收集Affinity官网数据或资料用于商业用途,对抄袭产品数据的行为我们将保留诉诸法律的权利。

产品相关数据会因产品批次、产品检测情况随时调整,如您已订购该产品,请以订购时随货说明书为准,否则请以官网内容为准,官网内容有改动时恕不另行通知。

Affinity保证所销售产品均经过严格质量检测。如您购买的商品在规定时间内出现问题需要售后时,请您在Affinity官方渠道提交售后申请。

产品仅供科学研究使用。不用于诊断和治疗。 

产品未经授权不得转售。

Affinity Biosciences将不会对在使用我们的产品时可能发生的专利侵权或其他侵权行为负责。Affinity Biosciences, Affinity Biosciences标志和所有其他商标所有权归Affinity Biosciences LTD.