NPHP1 antibody (Middle Region)

Catalog No. ABIN2786505

Product Details anti-NPHP1 Antibody

Target: NPHP1 (Nephronophthisis 1 (Juvenile) (NPHP1))

Binding Specificity: Middle Region

Reactivity: Human, Mouse, Rat, Dog, Cow, Guinea Pig, Horse, Pig, Rabbit, Zebrafish (Danio rerio)

Host: Rabbit

Clonality: Polyclonal

Conjugate: This NPHP1 antibody is un-conjugated

Application: Western Blotting (WB)

Sequence: GILFELGISY IRNSTGERGE LSCGWVFLKL FDASGVPIPA KTYELFLNGG

Predicted Reactivity: Cow: 100%, Dog: 100%, Guinea Pig: 93%, Horse: 100%, Human: 100%, Mouse: 100%, Pig: 100%, Rabbit: 100%, Rat: 100%, Zebrafish: 92%

Characteristics: This is a rabbit polyclonal antibody against NPHP1. It was validated on Western Blot using a cell lysate as a positive control.

Purification: Affinity Purified

Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human NPHP1

Application Details

Application Notes: Optimal working dilutions should be determined experimentally by the investigator.

Comment:

Antigen size: 733 AA

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: Lot specific

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: Avoid repeated freeze-thaw cycles.

Storage: -20 °C

Storage Comment: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Target Details for NPHP1

Target: NPHP1 (Nephronophthisis 1 (Juvenile) (NPHP1))

Alternative Name: NPHP1 (NPHP1 Products)

Synonyms: JBTS4 antibody, NPH1 antibody, SLSN1 antibody, nephrocystin-1 antibody, NPHP1 antibody, im:7162391 antibody, wu:fi59g07 antibody, zgc:152930 antibody, Nphp1 antibody, nephrocystin 1 antibody, nephronophthisis 1 (juvenile) homolog (human) antibody, nephronophthisis 1 (juvenile) L homeolog antibody, nephronophthisis 1 antibody, nephrocystin-1 antibody, NPHP1 antibody, Nphp1 antibody, nphp1.L antibody, nphp1 antibody, LOC100725987 antibody

Background: 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: MED28, CEP164, ADAM15, UBQLN4, TNK2, ARHGAP32, BCAR1, KHDRBS1, UBC, NPHP1, INVS, NPHP3, NPHP4, PAK2, FLNB, PTK2B, FLNA, TUBB, FLNC, TNS1,
Protein Size: 733

Molecular Weight: 83 kDa

Gene ID: 4867

NCBI Accession: NM_000272, NP_000263