产品货号 :  mlR11864

英文名称 :  KIRREL3

中文名称 :  肾病样蛋白3抗体

别    名 :  Kin of IRRE like 3 (Drosophila); Kin of IRRE like 3; Kin of irregular chiasm like protein 3 ; KIRRE; KIRREL 3; KIRREL-3; MRD4; NEPH2; Nephrin like 2; PRO19814; PRO4502; yUNQ5923; KIRR3_HUMAN.  

研究领域 :  细胞生物  神经生物学  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :   Human, Mouse, Rat, Chicken,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  83kDa

细胞定位 :  细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human KIRREL3:561-450/778 <Extracellular>

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

产品介绍 :   NEPH2 is a 778 amino acid single-pass type I membrane protein that belongs to the nephrin-like protein family and immunoglobulin superfamily. Expressed in both fetal and adult brain, as well as podocytes of kidney glomeruli, NEPH2 contains five Ig-like C2-type (immunoglobulin-like) domains and is thought to plaly a role in the hematopoetic supportive capacity of stroma cells. NEPH2 undergoes alternative splicing to produce two isoforms and contains a C-terminal cytoplasmic domain which it uses to interact with Podocin, a podocyte protein involved in ultrafiltration. Defects in the gene encoding NEPH2 are associated with mental retardation autosomal dominant type 4 (MRD4).

Function:

NEPH2 is a member of the nephrin like protein family, which includes NEPH1 (KIRREL; MIM 607428) and NEPH3 (KIRREL2; MIM 607762). The cytoplasmic domains of these proteins interact with the C terminus of podocin (NPHS2; MIM 604766), and the genes are expressed in kidney podocytes, cells involved in ensuring size and charge selective ultrafiltration (Sellin et al., 2003 [PubMed 12424224]).

Subunit:

Interacts with the C-terminus of NPHS2/podocin. Interacts with CASK.

Subcellular Location:

Cell membrane; Single-pass type I membrane protein

Tissue Specificity:

Expressed in fetal and adult brain. Also expressed in kidney, specifically in podocytes of kidney glomeruli.

DISEASE:

Note=A chromosomal aberration involving KIRREL3 and CDH15 is found in a patient with severe mental retardation and dysmorphic facial features. Translocation t(11;16)(q24.2;q24).

Defects in KIRREL3 are the cause of mental retardation autosomal dominant type 4 (MRD4) [MIM:612581]. Mental retardation is characterized by significantly sub-average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period.

Similarity:

Belongs to the immunoglobulin superfamily.

Contains 5 Ig-like C2-type (immunoglobulin-like) domains.

SWISS:

Q8IZU9

Gene ID:

84623

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片