Name: Anti-LEPRE1 Antibody (CAB10322)
Brand: Cell Biology Antibodies 1
SKU: CAB10322
Availability: OutOfStock

Antibody Name:	Anti-LEPRE1 Antibody
Antibody SKU:	CAB10322
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 497-736 of human LEPRE1 (NP_071751.3).

Application:	WB
Recommended Dilution:	WB 1:1000 - 1:2000
Reactivity:	Human, Mouse
Positive Samples:	A375, NCI-H460, HT-29, HeLa, SKOV3

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 497-736 of human LEPRE1 (NP_071751.3).
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:	GDGY RGQT SPHT PNEK FYGV TVFK ALKL GQEG KVPL QSAH LYYN VTEK VRRI MESY FRLD TPLY FSYS HLVC RTAI EEVQ AERK DDSH PVHV DNCI LNAE TLVC VKEP PAYT FRDY SAIL YLNG DFDG GNFY FTEL DAKT VTAE VQPQ CGRA VGFS SGTE NPHG VKAV TRGQ RCAI ALWF TLDP RHSE RDRV QADD LVKM LFSP EEMD LSQE QPLD AQQG PPEP AQES LSGS ESKP KDEL
Gene ID:	64175
Uniprot:	Q32P28
Cellular Location:	Endoplasmic reticulum, Secreted, extracellular matrix, extracellular space
Calculated MW:	41kDa/78kDa/83kDa/90kDa
Observed MW:	100kDa

Synonyms:

P3H1, GROS1, LEPRE1, OI8

Background:

This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Three alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined.

UniProt Protein Function:	LEPRE1: Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro- Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts. Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8). A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest. Belongs to the leprecan family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:EC 1.14.11.7; Secreted, signal peptide; Oxidoreductase; Cell development/differentiation; Secreted Chromosomal Location of Human Ortholog: 1p34.1 Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; membrane Molecular Function:collagen binding; procollagen-proline 3-dioxygenase activity; protein complex binding Biological Process: collagen metabolic process; protein amino acid hydroxylation; protein folding; protein stabilization; regulation of protein secretion Disease: Osteogenesis Imperfecta, Type Viii
NCBI Summary:	This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Three alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined. [provided by RefSeq, Aug 2011]
UniProt Code:	Q32P28
NCBI GenInfo Identifier:	109892809
NCBI Gene ID:	64175
NCBI Accession:	Q32P28.2
UniProt Secondary Accession:	Q32P28,Q7KZR4, Q96BR8, Q96SK8, Q96SL5, Q96SN3, Q9H6K3 Q9HC86, Q9HC87,
UniProt Related Accession:	Q32P28
Molecular Weight:	78,922 Da
NCBI Full Name:	Prolyl 3-hydroxylase 1
NCBI Synonym Full Names:	prolyl 3-hydroxylase 1
NCBI Official Symbol:	P3H1
NCBI Official Synonym Symbols:	OI8; GROS1; LEPRE1
NCBI Protein Information:	prolyl 3-hydroxylase 1
UniProt Protein Name:	Prolyl 3-hydroxylase 1
UniProt Synonym Protein Names:	Growth suppressor 1
UniProt Gene Name:	P3H1
UniProt Entry Name:	P3H1_HUMAN

Western blot analysis of extracts of various cell lines, using LEPRE1 antibody (CAB10322) 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: 30s.

ELISA Kits

Human P3H1 (Prolyl 3-hydroxylase 1) ELISA Kit (HUFI05119)

Secondary Antibody

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