NPHP1 Antikörper (Middle Region)
Kurzübersicht für NPHP1 Antikörper (Middle Region) (ABIN2786505)
Target
Alle NPHP1 Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
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Bindungsspezifität
- Middle Region
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Verwendungszweck
- NPHP1 Antibody - middle region
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Sequenz
- GILFELGISY IRNSTGERGE LSCGWVFLKL FDASGVPIPA KTYELFLNGG
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Homologie
- Cow: 100%, Dog: 100%, Guinea Pig: 93%, Horse: 100%, Human: 100%, Mouse: 100%, Pig: 100%, Rabbit: 100%, Rat: 100%, Zebrafish: 92%
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Produktmerkmale
- This is a rabbit polyclonal antibody against NPHP1. It was validated on Western Blot using a cell lysate as a positive control.
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Aufreinigung
- Affinity Purified
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Immunogen
- The immunogen is a synthetic peptide directed towards the middle region of human NPHP1
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Applikationshinweise
- Optimal working dilution should be determined by the investigator.
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Kommentare
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We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP56084-100UG
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Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Format
- Liquid
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Konzentration
- 0.5 mg/mL
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Buffer
- Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
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Konservierungsmittel
- Sodium azide
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Vorsichtsmaßnahmen
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Handhabung
- prevent freeze-thaw cycles
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Lagerung
- 4 °C,-20 °C
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Informationen zur Lagerung
- For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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- NPHP1 (Nephronophthisis 1 (Juvenile) (NPHP1))
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Andere Bezeichnung
- NPHP1
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Hintergrund
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Background Information: 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.
Gene Name: Nephronophthisis 1 (juvenile)
Alternative Symbols: NPH1, JBTS4, SLSN1
Protein Name: Nephrocystin-1
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Molekulargewicht
- 83kDa
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Gen-ID
- 4867
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NCBI Accession
- NP_000263
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UniProt
- O15259
Target
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