Antibody Name:	Anti-HADHB Antibody
Antibody SKU:	CAB5716
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 34-270 of human HADHB (NP_000174.1).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:10 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	BT-474, SW480, HepG2, 293T, Jurkat, Mouse liver, Mouse kidney, Mouse heart, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 34-270 of human HADHB (NP_000174.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	AAPA VQTK TKKT LAKP NIRN VVVV DGVR TPFL LSGT SYKD LMPH DLAR AALT GLLH RTSV PKEV VDYI IFGT VIQE VKTS NVAR EAAL GAGF SDKT PAHT VTMA CISA NQAM TTGV GLIA SGQC DVIV AGGV ELMS DVPI RHSR KMRK LMLD LNKA KSMG QRLS LISK FRFN FLAP ELPA VSEF STSE TMGH SADR LAAA FAVS RLEQ DEYA LRSH SLAK KAQD EGLL SDVV PFKV P
Gene ID:	3032
Uniprot:	P55084
Cellular Location:	Endoplasmic reticulum, Mitochondrion, Mitochondrion inner membrane, Mitochondrion outer membrane
Calculated MW:	48kDa/51kDa
Observed MW:	51kDa

Synonyms:

HADHB, ECHB, MSTP029, MTPB, TP-BETA

Background:

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. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described.

UniProt Protein Function:	HADHB: Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency). 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. Belongs to the thiolase family.
UniProt Protein Details:	Protein type:Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Transferase; Lipid Metabolism - fatty acid elongation in mitochondria; EC 2.3.1.16 Chromosomal Location of Human Ortholog: 2p23 Cellular Component: mitochondrial envelope; mitochondrial outer membrane; mitochondrion; endoplasmic reticulum; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex Molecular Function:protein binding; acyl-CoA binding; enoyl-CoA hydratase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; NAD binding; acetyl-CoA C-acyltransferase activity; 3-hydroxyacyl-CoA dehydrogenase activity Biological Process: fatty acid beta-oxidation; phospholipid metabolic process; glycerophospholipid biosynthetic process; cellular lipid metabolic process Disease: Trifunctional Protein Deficiency
NCBI Summary:	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. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013]
UniProt Code:	P55084
NCBI GenInfo Identifier:	116241345
NCBI Gene ID:	3032
NCBI Accession:	P55084.3
UniProt Secondary Accession:	P55084,O14969, Q53TA6, Q96C77, Q9H3F5, Q9T2V8, B2RB16 B4E2W0,
UniProt Related Accession:	P55084
Molecular Weight:	474
NCBI Full Name:	Trifunctional enzyme subunit beta, mitochondrial
NCBI Synonym Full Names:	hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
NCBI Official Symbol:	HADHB
NCBI Official Synonym Symbols:	ECHB; MTPB; MSTP029; TP-BETA
NCBI Protein Information:	trifunctional enzyme subunit beta, mitochondrial; beta-ketothiolase; acetyl-CoA acyltransferase; 2-enoyl-Coenzyme A (CoA) hydratase, beta subunit; 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit
UniProt Protein Name:	Trifunctional enzyme subunit beta, mitochondrial
UniProt Synonym Protein Names:	TP-betaIncluding the following 1 domains:3-ketoacyl-CoA thiolase (EC:2.3.1.16)Alternative name(s):Acetyl-CoA acyltransferase; Beta-ketothiolase
Protein Family:	Trifunctional enzyme
UniProt Gene Name:	HADHB
UniProt Entry Name:	ECHB_HUMAN

	Western blot analysis of extracts of various cell lines, using HADHB antibody (CAB5716) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s.
	Immunofluorescence analysis of A549 cells using HADHB antibody (CAB5716). Blue: DAPI for nuclear staining.