HADHB Antilichaam - C-terminaal gebied: Biotine (ARP48133_P050-Biotine)

Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit

ECHB, MTPB, MSTP029, TP-BETA

3032

P55084

NP_000174

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Pig, Rabbit, Yeast, Zebrafish

The immunogen is a synthetic peptide directed towards the C terminal region of human HADHB

HADHB is 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 HADHB gene result in trifunctional protein deficiency. The protein can also bind RNA and decreases the stability of some mRNAs.This 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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

HUWE1; TP53; TUBG1; SUMO2; SUMO3; UBC; PARK2; PPP6R1; ADRB2; CSNK2A2; PAN2; ATF2; vpu; TIMM8B; COX17; IQCB1; SUOX; CHCHD4; TUBB1; RPS8; OPA1; HSP90AB1; HADHA; DES; GRK5; FBXO6; CDK2; LEO1; RTF1; TK1; SMN1; PSMA3; GRB7; RCC1; CDKN1A; ANXA7; Edc4; MYC; SQST

Biotin

Polyclonal Antibody

WB, IHC

Affinity Purified

HADHB is supported by BioGPS gene expression data to be expressed in HepG2

0.5 mg/ml

Cow: 93%; Dog: 100%; Guinea Pig: 93%; Horse: 93%; Human: 100%; Mouse: 100%; Pig: 100%; Rabbit: 93%; Rat: 100%; Yeast: 83%; Zebrafish: 86%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

47kDa

Wang, R., (2006) Zhonghua Fu Chan Ke Za Zhi 41 (10), 672-675

Wet Ice

474

HADHB

HADHB

Rabbit

Trifunctional enzyme subunit beta, mitochondrial

NM_000183

LLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAE

Beta, mitochondrial