Anti-EHHADH 항체 (CAB13488)

Antibody Name:	Anti-EHHADH Antibody
Antibody SKU:	CAB13488
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 444-723 of human EHHADH (NP_001957.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	HepG2, Mouse liver, Rat liver, Rat kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 444-723 of human EHHADH (NP_001957.2).
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:	EVIP SQYS SPTT IATV MNLS KKIK KIGV VVGN CFGF VGNR MLNP YYNQ AYFL LEEG SKPE EVDQ VLEE FGFK MGPF RVSD LAGL DVGW KSRK GQGL TGPT LLPG TPAR KRGN RRYC PIPD VLCE LGRF GQKT GKGW YQYD KPLG RIHK PDPW LSKF LSRY RKTH HIEP RTIS QDEI LERC LYSL INEA FRIL GEGI AASP EHID VVYL HGYG WPRH KGGP MFYA STVG LPTV LEKL QKYY RQNP DIPQ LEPS DYLK KLAS QGNP PLKE WQSL AGSP SSKL
Gene ID:	1962
Uniprot:	Q08426
Cellular Location:	Peroxisome
Calculated MW:	69kDa/79kDa
Observed MW:	79kDa

Synonyms:

EHHADH, ECHD, FRTS3, L-PBE, LBFP, LBP, PBFE

Background:

The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	EHHADH: 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Amino Acid Metabolism - lysine degradation; EC 5.3.3.8; Oxidoreductase; EC 4.2.1.17; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid; EC 1.1.1.35; Mitochondrial; Lyase; Isomerase; Secondary Metabolites Metabolism - limonene and pinene degradation; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - propanoate Chromosomal Location of Human Ortholog: 3q26.3-q28 Cellular Component: mitochondrion; peroxisome; cytosol Molecular Function:enzyme binding; dodecenoyl-CoA delta-isomerase activity; enoyl-CoA hydratase activity; coenzyme binding; 3-hydroxyacyl-CoA dehydrogenase activity; receptor binding Biological Process: fatty acid beta-oxidation; internal protein amino acid acetylation Disease: Fanconi Renotubular Syndrome 3
NCBI Summary:	The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
UniProt Code:	Q08426
NCBI GenInfo Identifier:	223590229
NCBI Gene ID:	1962
NCBI Accession:	Q08426.3
UniProt Secondary Accession:	Q08426,Q58EZ5, A8K6Y3, B4DWG3, D3DNU0,
UniProt Related Accession:	Q08426
Molecular Weight:	723
NCBI Full Name:	Peroxisomal bifunctional enzyme
NCBI Synonym Full Names:	enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase
NCBI Official Symbol:	EHHADH
NCBI Official Synonym Symbols:	LBP; ECHD; LBFP; PBFE; FRTS3; L-PBE
NCBI Protein Information:	peroxisomal bifunctional enzyme; PBE; 3,2-trans-enoyl-CoA isomerase; peroxisomal enoyl-CoA hydratase; L-3-hydroxyacyl-CoA dehydrogenase; L-bifunctional protein, peroxisomal; enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase
UniProt Protein Name:	Peroxisomal bifunctional enzyme
Protein Family:	Peroxisomal bifunctional enzyme
UniProt Gene Name:	EHHADH
UniProt Entry Name:	ECHP_HUMAN

	Western blot analysis of extracts of various cell lines, using EHHADH antibody (CAB13488) 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 Basic Kit (RM00020). Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded rat kidney using EHHADH antibody (CAB13488) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human liver damage using EHHADH antibody (CAB13488) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of A549 cells using EHHADH antibody (CAB13488). Blue: DAPI for nuclear staining.

Antibodies

Anti-EHHADH Antibody (CAB5717)

EHHADH Antibody (PACO06737)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)