Item no. |
E-AB-53023-120 |
Manufacturer |
Elabscience
|
Amount |
120uL |
Category |
|
Type |
Antibody Polyclonal |
Applications |
WB, IHC, ELISA |
Specific against |
Human, Mouse |
Host |
Rabbit |
Isotype |
IgG |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
JBTS4, Juvenile nephronophthisis 1 protein, Nephrocystin 1, nephronophthisis 1 (juvenile), Nephronophthisis, NPH1, NPHP1, SLSN1 |
Similar products |
NPHP1, NPH1, JBTS4, SLSN1, Nephrocystin 1, Juvenile nephronophthisis 1 protein, nephronophthisis 1 (juvenile), Nephronophthisis |
Available |
|
Research Areas |
Cell Biology, Signal transduction |
Synonyms |
JBTS4, Juvenile nephronophthisis 1 protein, Nephrocystin 1, nephronophthisis 1 (juvenile), Nephronophthisis, NPH1, NPHP1, SLSN1 |
Swissprot |
O15259 |
Background |
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. |
Concentration |
1.02 mg/mL |
Storage |
Store at -20C. Avoid freeze / thaw cycles. |
Immunogen |
Fusion protein of human NPHP1 |
Buffer |
PBS with 0.05% NaN3 and 40% Glycerol, pH7.4 |
Purification Method |
Antigen affinity purification |
Dilution |
WB 1:500-1:2000, IHC 1:50-1:300, ELISA 1:5000-1:10000 |
Conjugation |
Unconjugated |
Calculated MW |
83 kDa |
Observed MW |
Refer to figures |
Gene Accession |
BC009789 |
Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.
All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.