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Product Name KIRREL3 Chinese Name 肾病样蛋白3抗体 Alias 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. Research Area Cell biology Neurobiology Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Chicken, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1μg/Test ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 83kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human KIRREL3: 351-450/778 <Extracellular> Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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
Database links:Entrez Gene: 84623 Human
Entrez Gene: 67703 Mouse
Omim: 607761 Human
SwissProt: Q8IZU9 Human
SwissProt: Q8BR86 Mouse
Unigene: 376015 Human
Unigene: 220710 Mouse
Unigene: 7602 Rat
Product Picture Blank control(blue):Mouse nephrocytes (fixed with 2% paraformaldehyde (10 min)).
Primary Antibody:Rabbit Anti- KIRREL3 antibody(SL11864R), Dilution: 1μg in 100 μL 1X PBS containing 0.5% BSA;
Isotype Control Antibody: Rabbit IgG(orange) ,used under the same conditions );
Secondary Antibody: Goat anti-rabbit IgG-PE(white blue), Dilution: 1:200 in 1 X PBS containing 0.5% BSA.
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