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NPHP1 Antibody - middle region

CAT: 0247-ARP56084_P050-25ULSize: 25 µLDry Ice: NoHazardous: No
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CAT#:0247-ARP56084_P050-25ULSize:25 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Description
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CAS Number
9007-83-4
Gene Name
Nephronophthisis 1 (juvenile)
Gene Aliases
NPH1, JBTS4, SLSN1
Gene ID
4867
Swiss Prot
O15259-4
Accession Number
NP_000263
Reactivity
Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Pig, Rabbit, Zebrafish
Immunogen
The immunogen is a synthetic peptide directed towards the middle region of human NPHP1
Target
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.
Partner Proteins
MED28; CEP164; ADAM15; UBQLN4; TNK2; ARHGAP32; BCAR1; KHDRBS1; UBC; NPHP1; INVS; NPHP3; NPHP4; PAK2; FLNB; PTK2B; FLNA; TUBB; FLNC; TNS1
Clonality
Polyclonal
Type
Polyclonal Antibody
Sequence
GILFELGISYIRNSTGERGELSCGWVFLKLFDASGVPIPAKTYELFLNGG
Applications
WB
Purification
Affinity Purified
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol
Concentration
0.5 mg/ml
Homology
Cow: 100%; Dog: 100%; Guinea Pig: 93%; Horse: 100%; Human: 100%; Mouse: 100%; Pig: 100%; Rabbit: 100%; Rat: 100%; Zebrafish: 92%
Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Reconstitution
For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
Molecular Weight
83kDa
Protein Length
733
NCBI Gene Symbol
NPHP1
Host or Source
Rabbit
Protein Name
Nephrocystin-1
Gene Name URL
NPHP1
Nucleotide Accession Number
NM_000272

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