Antibody Name:	Anti-XPNPEP3 Antibody
Antibody SKU:	CAB14919
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 1-250 of human XPNPEP3 (NP_071381.1).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	A-549, A-431, 293T, Raji, Mouse kidney, Mouse heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human XPNPEP3 (NP_071381.1).
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:	MPWL LSAP KLVP AVAN VRGL SGCM LCSQ RRYS LQPV PERR IPNR YLGQ PSPF THPH LLRP GEVT PGLS QVEY ALRR HKLM SLIQ KEAQ GQSG TDQT VVVL SNPT YYMS NDIP YTFH QDNN FLYL CGFQ EPDS ILVL QSLP GKQL PSHK AILF VPRR DPSR ELWD GPRS GTDG AIAL TGVD EAYT LEEF QHLL PKMK AETN MVWY DWMR PSHA QLHS DYMQ PLTE AKAK SKNK VRGV QQLI QRLR LIKS PA
Gene ID:	63929
Uniprot:	Q9NQH7
Cellular Location:	Mitochondrion
Calculated MW:	31kDa/32kDa/48kDa/54kDa/57kDa
Observed MW:	57kDa

Synonyms:

XPNPEP3, APP3, ICP55, NPHPL1

Background:

The protein encoded by this gene belongs to the family of X-pro-aminopeptidases that utilize a metal cofactor, and remove the N-terminal amino acid from peptides with a proline residue in the penultimate position. This protein has been shown to localize to the mitochondria of renal cells, and have a role in ciliary function. Mutations in this gene are associated with nephronophthisis-like nephropathy-1. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene, however, expression of some of these isoforms in vivo is not known.

UniProt Protein Function:	XPNPEP3: Defects in XPNPEP3 are the cause of nephronophthisis-like nephropathy type 1 (NPHPL1). A disorder with features of nephronophthisis, a cystic kidney disease leading to end-stage renal failure. Nephronophthisis is histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. Typical clinical manifestation are chronic renal failure, anemia, polyuria, polydipsia, isosthenuria, and growth retardation. Associations with extrarenal symptoms are frequent. In NPHPL1 patients, extrarenal symptoms include hypertension, essential tremor, sensorineural hearing loss and gout. Severely affected individuals can manifest a mitochondrial disorder with isolated complex I deficiency activity in muscle, seizures, mental retardation and hypertrophic dilated cardiomyopathy. Belongs to the peptidase M24B family. 5 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Protease; EC 3.4.11.9 Chromosomal Location of Human Ortholog: 22q13.2 Cellular Component: mitochondrion Molecular Function:aminopeptidase activity Biological Process: glomerular filtration; protein processing Disease: Nephronophthisis-like Nephropathy 1
NCBI Summary:	The protein encoded by this gene belongs to the family of X-pro-aminopeptidases that utilize a metal cofactor, and remove the N-terminal amino acid from peptides with a proline residue in the penultimate position. This protein has been shown to localize to the mitochondria of renal cells, and have a role in ciliary function. Mutations in this gene are associated with nephronophthisis-like nephropathy-1. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene, however, expression of some of these isoforms in vivo is not known.[provided by RefSeq, Mar 2011]
UniProt Code:	Q9NQH7
NCBI GenInfo Identifier:	74761652
NCBI Gene ID:	63929
NCBI Accession:	Q9NQH7.1
UniProt Secondary Accession:	Q9NQH7,Q6I9V9, Q8NDA6, Q9BV27, Q9BVH0, B2R9G1, B7Z790 B7Z7B2,
UniProt Related Accession:	Q9NQH7
Molecular Weight:	32,712 Da
NCBI Full Name:	Probable Xaa-Pro aminopeptidase 3
NCBI Synonym Full Names:	X-prolyl aminopeptidase 3
NCBI Official Symbol:	XPNPEP3
NCBI Official Synonym Symbols:	APP3; ICP55; NPHPL1
NCBI Protein Information:	probable Xaa-Pro aminopeptidase 3
UniProt Protein Name:	Probable Xaa-Pro aminopeptidase 3
UniProt Synonym Protein Names:	Aminopeptidase P3; APP3
Protein Family:	Probable Xaa-Pro aminopeptidase
UniProt Gene Name:	XPNPEP3
UniProt Entry Name:	XPP3_HUMAN

	Western blot analysis of extracts of various cell lines, using XPNPEP3 Rabbit pAb (CAB14919) 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: 5s.
	Immunofluorescence analysis of L929 cells using XPNPEP3 antibody (CAB14919) at dilution of 1:100. Blue: DAPI for nuclear staining.