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Mouse Anti-KIRREL1 Recombinant Antibody (G-8) (CBMAB-N0886-WJ)

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Basic Information

Host Animal
Mouse
Clone
G-8
Application
WB, IP, IF, ELISA
Specificity
Mouse, Rat, Human
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
More Infomation

Target

Full Name
Kirre Like Nephrin Family Adhesion Molecule 1
Introduction
NEPH1 is a member of the nephrin-like protein family, which includes NEPH2 (MIM 607761) and NEPH3 (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]).[supplied by OMIM, Mar 2008]
Entrez Gene ID
Human55243
Rat310695
UniProt ID
HumanQ96J84
MouseQ80W68
RatQ6X936
Alternative Names
Kirre Like Nephrin Family Adhesion Molecule 1; Nephrin-Like Protein 1; Kin Of Irregular Chiasm-Like Protein 1; KIRREL; NEPH1; Kin Of IRRE Like (Drosophila); Kin Of IRRE-Like Protein 1; Kin Of IRRE Like; Nephrin Related;
Function
Required for proper function of the glomerular filtration barrier. It is involved in the maintenance of a stable podocyte architecture with interdigitating foot processes connected by specialized cell-cell junctions, known as the slit diaphragm (PubMed:31472902).
It is a signaling protein that needs the presence of TEC kinases to fully trans-activate the transcription factor AP-1 (By similarity).
Biological Process
Cell-cell adhesionManual Assertion Based On ExperimentIBA:GO_Central
Cell-cell junction maintenanceManual Assertion Based On ExperimentIMP:UniProtKB
ExcretionIEA:Ensembl
Glomerular filtrationManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of protein phosphorylationIEA:Ensembl
Positive regulation of actin filament polymerizationIEA:Ensembl
Cellular Location
Cell membrane. Predominantly located at podocyte slit diaphragm.
Involvement in disease
Nephrotic syndrome 23 (NPHS23):
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS23 is an autosomal recessive form characterized by onset of proteinuria in the first or second decade of life, and variable outcome. Some patients have normal renal function after many years, whereas others may progress to chronic kidney disease.
Topology
Extracellular: 17-499
Helical: 500-520
Cytoplasmic: 521-757
PTM
Phosphorylation probably regulates the interaction with NSH2. Phosphorylated at Tyr-605 and Tyr-606 by FYN, leading to GRB2 binding (By similarity).
N-glycosylated.

Paul, A., Annunziato, S., Lu, B., Sun, T., Evrova, O., Planas-Paz, L., ... & Cong, F. (2022). Cell adhesion molecule KIRREL1 is a feedback regulator of Hippo signaling recruiting SAV1 to cell-cell contact sites. Nature Communications, 13(1), 930.

Gu, Y., Wang, Y., Sha, Z., He, C., Zhu, Y., Li, J., ... & Yu, F. X. (2022). Transmembrane protein KIRREL1 regulates Hippo signaling via a feedback loop and represents a therapeutic target in YAP/TAZ-active cancers. Cell Reports, 40(9).

Solanki, A. K., Widmeier, E., Arif, E., Sharma, S., Daga, A., Srivastava, P., ... & Nihalani, D. (2019). Mutations in KIRREL1, a slit diaphragm component, cause steroid-resistant nephrotic syndrome. Kidney international, 96(4), 883-889.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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