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Product Name KIRREL1 Chinese Name 肾病样蛋白1抗体 Alias Kin of IRRE like (Drosophila); Kin of IRRE like; Kin of IRRE like protein 1; Kin of IRRE-like protein 1; Kin of irregular chiasm like protein 1; Kin of irregular chiasm-like protein 1; KIRR1_HUMAN; Kirrel; MGC129542; MGC129543; NEPH 1; NEPH1; Nephrin like protein 1; Nephrin related; Nephrin-like protein 1; KIRR1_HUMAN. Research Area Cell biology immunology Developmental biology Kinases and Phosphatases Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=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 82kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human KIRREL1/NEPH1: 301-400/757 <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 Neph1(Kirrel) belongs to a family of three closely related transmembrane proteins of the Ig superfamily with a structure similar to that of nephrin. Neph1 consists of a signal peptide, five Ig-like C2-type domains with the middle domain overlapping with a PKD-like domain, an RGD sequence, a transmembrane domain and a cytoplasmic tail. Neph1 is abundantly expressed in the kidney, specifically expressed in podocytes of kidney glomeruli, and plays a significant role in the normal development and function of the glomerular permeability. Neph1 interacts with nephrin in vitro and in vivo, and able to stimulate transcriptional activation in a model system, such as the activation the transcription factor AP-1 via the stimulation of a MAPK module. Neph1 is crucial for the integrity of the slit diaphragm, as Neph1 gene knockout mice results in effacement of glomerular podocytes, heavy proteinuria, and early postnatal death. .
Tissue specificity:
Abundantly expressed in kidney. Specifically expressed in podocytes of kidney glomeruli.
Function:
Plays a significant role in the normal development and function of the glomerular permeability. Signaling protein that needs the presence of TEC kinases to fully trans-activate the transcription factor AP-1 (By similarity).
Subunit:
Interacts with TJP1/ZO-1 and with NPHS2/podocin (via the C-terminus). Interacts with NPHS1/nephrin (via the Ig-like domains); this interaction is dependent on KIRREL glycosylation. Homodimer (via the Ig-like domains). Interacts when tyrosine-phosphorylated with GRB2 (By similarity).
Subcellular Location:
Cell membrane; Single-pass type I membrane protein.
Tissue Specificity:
Abundantly expressed in kidney. Specifically expressed in podocytes of kidney glomeruli.
Post-translational modifications:
Phosphorylation probably regulates the interaction with NSH2. Phosphorylated at Tyr-605 and Tyr-606 by FYN, leading to GRB2 binding (By similarity).
N-glycosylated (By similarity).
Similarity:
Belongs to the immunoglobulin superfamily.
Contains 5 Ig-like C2-type (immunoglobulin-like) domains.
SWISS:
Q96J84
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
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