Rabbit Anti-HADHB/AF350 Conjugated antibody
2 enoyl Coenzyme A (CoA) hydratase beta subunit; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit; 3 ketoacyl Coenzyme A thiolase; 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit;
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Product Name Anti-HADHB/AF350 Chinese Name AF350标记的羟辅酶A脱氢酶β抗体 Alias 2 enoyl Coenzyme A (CoA) hydratase beta subunit; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit; 3 ketoacyl Coenzyme A thiolase; 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit; Acetyl CoA acyltransferase; Beta ketothiolase; ECHB; HADH; Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase; Hydroxyacyl Coenzyme A dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit; Mitochondrial trifunctional enzyme beta subunit; Mitochondrial trifunctional protein beta subunit; MTPB; TP beta. TPbeta; Trifunctional enzyme subunit beta; Trifunctional enzyme subunit beta mitochondrial; Trifunctional protein; ECHB_HUMAN. Research Area Tumour Cell biology immunology transcriptional regulatory factor Kinases and Phosphatases Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep, ) Applications IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 47kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human HADHB Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
The HADHB gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Alternatively spliced transcript variants have been found; however, their full-length nature is not known.
Subunit:
Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits. Interacts with RSAD2/viperin.
Subcellular Location:
Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum.
DISEASE:
Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.
Similarity:
Belongs to the thiolase family.
Database links:
UniProtKB/Swiss-Prot: P55084.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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| Username | Quantity | bought time |
| Na*** | 2 | 2024-04-16 |
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