Nephronophthisis 1 (Juvenile) (NPHP1) (Middle Region) Peptide
NPHP1
Reactivity: Human
Host: Synthetic
BP, WB
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- Target See all NPHP1 products
- NPHP1 (Nephronophthisis 1 (Juvenile) (NPHP1))
- Protein Region
- Middle Region
- Origin
- Human
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Source
- Synthetic
- Application
- Blocking Peptide (BP), Western Blotting (WB)
- Characteristics
- This is a synthetic peptide designed for use in combination with anti-NPHP1 antibody (Catalog #: ARP56084_P050). It may block above mentioned antibody from binding to its target protein in western blot and/or immunohistochecmistry under proper experimental settings. There is no guarantee for its use in other applications.
- Purification
- Purified
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- Application Notes
- Each Investigator should determine their own optimal working dilution for specific applications.
- Restrictions
- For Research Use only
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- Format
- Lyophilized
- Reconstitution
- Add 100 μL of sterile PBS. Final peptide concentration is 1 mg/mL in PBS.
- Concentration
- 1 mg/mL
- Buffer
- Final peptide concentration is 1 mg/mL in PBS.
- Handling Advice
- Avoid repeated freeze-thaw cycles.
- Storage
- -20 °C
- Storage Comment
- For longer periods of storage, store at -20°C. Avoid repeat freeze-thaw cycles.
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- Target
- NPHP1 (Nephronophthisis 1 (Juvenile) (NPHP1))
- Background
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Together with Cas NPHP1 may play a role in the control of epithelial cell polarity. NPHP1 seems to help to recruit protein tyrosine kinase 2 beta (PTK2B) to cell matrix adhesions, thereby initiating phosphorylation of PTK2B and PTK2B-dependent signaling.This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding different isoforms have been found for this gene.
Alias Symbols: JBTS4, NPH1, SLSN1
Protein Interaction Partner: ADAM15,BCAR1,FLNA,FLNB,FLNC,INVS,NPHP1,NPHP3,NPHP4,PAK2,PTK2B,TUBB,UBQLN4,BCAR1,FLNA,FLNB,INVS,NPHP1,NPHP3,NPHP4,PTK2B,TNS1,TUBB
Protein Size: 733 - Molecular Weight
- 83 kDa
- Gene ID
- 4867
- NCBI Accession
- NM_000272, NP_000263
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