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anti-NPHP4 antibody :: nephronophthisis 4 Polyclonal Antibody

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Catalog # MBS719341
Unit / Price
  0.05 mL  /  $230 +1 FREE 8GB USB
  0.15 mL  /  $500 +1 FREE 8GB USB
anti-NPHP4 antibody
Product Name

nephronophthisis 4 (NPHP4), Polyclonal Antibody

Full Product Name

Rabbit anti-human nephronophthisis 4 polyclonal Antibody

Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
607215
Clonality
Polyclonal
Isotype
IgG
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Immunogen
Human NPHP4
Storage Buffer
PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-49244 / sc-49246 / sc-49247 / sc-99012
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of anti-NPHP4 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Applications Tested/Suitable for anti-NPHP4 antibody
ELISA (EIA), Immunofluorescence (IF)
NCBI/Uniprot data below describe general gene information for NPHP4. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
99,953 Da[Similar Products]
NCBI Official Full Name
nephronophthisis 4
NCBI Official Synonym Full Names
nephronophthisis 4
NCBI Official Symbol
NPHP4  [Similar Products]
NCBI Official Synonym Symbols
POC10; SLSN4
  [Similar Products]
NCBI Protein Information
nephrocystin-4; nephroretinin; POC10 centriolar protein homolog
UniProt Protein Name
Nephrocystin-4
UniProt Synonym Protein Names
Nephroretinin
Protein Family
UniProt Gene Name
NPHP4  [Similar Products]
UniProt Synonym Gene Names
KIAA0673  [Similar Products]
UniProt Entry Name
NPHP4_HUMAN
NCBI Summary for NPHP4
This gene encodes a protein involved in renal tubular development and function. This protein interacts with nephrocystin, and belongs to a multifunctional complex that is localized to actin- and microtubule-based structures. Mutations in this gene are associated with nephronophthisis type 4, a renal disease, and with Senior-Loken syndrome type 4, a combination of nephronophthisis and retinitis pigmentosa. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2014]
UniProt Comments for NPHP4
NPHP4: Involved in the organization of apical junctions in kidney cells together with NPHP1 and RPGRIP1L/NPHP8. Does not seem to be strictly required for ciliogenesis. Defects in NPHP4 are the cause of nephronophthisis type 4 (NPHP4); also known as familial juvenile nephronophthisis 4. NPHP4 is an autosomal recessive inherited disease resulting in end-stage renal disease at age ranging between 6 and 35 years. It is a progressive tubulo-interstitial kidney disorder characterized by polydipsia, polyuria, anemia and growth retardation. The most prominent histological features are modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. Ciliary dysfunction leads to a broad spectrum of disorders, collectively termed ciliopathies. Overlapping clinical features include retinal degeneration, renal cystic disease, skeletal abnormalities, fibrosis of various organ, and a complex range of anatomical and functional defects of the central and peripheral nervous system. The ciliopathy range of diseases includes Meckel-Gruber syndrome, Bardet-Biedl syndrome, Joubert syndrome, nephronophtisis, Senior-Loken syndrome, and Jeune asphyxiating thoracic dystrophy among others. Single-locus allelism is insufficient to explain the variable penetrance and expressivity of such disorders, leading to the suggestion that variations across multiple sites of the ciliary proteome, including NPHP4, influence the clinical outcome. Defects in NPHP4 are the cause of Senior-Loken syndrome type 4 (SLSN4). SLSN is a renal-retinal disorder characterized by progressive wasting of the filtering unit of the kidney, with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life. Belongs to the NPHP4 family.

Protein type: Cytoskeletal

Chromosomal Location of Human Ortholog: 1p36

Cellular Component: centrosome; tight junction; membrane; intercellular junction; cytosol; photoreceptor connecting cilium

Molecular Function: protein binding; structural molecule activity

Biological Process: cell-cell adhesion; sperm motility; retina development in camera-type eye; organelle organization and biogenesis; photoreceptor cell maintenance; actin cytoskeleton organization and biogenesis; signal transduction; visual behavior

Disease: Nephronophthisis 4; Senior-loken Syndrome 4
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

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Pathways associated with anti-NPHP4 antibody
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