Product Name
NPHP3, siRNA
Full Product Name
NPHP3 siRNA (Human)
Product Synonym Names
KIAA2000; Nephrocystin-3
Product Gene Name
NPHP3 sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q7Z494
Specificity
NPHP3 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Purity/Purification
> 97%
Form/Format
Lyophilized powder
Quality Control
Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
Directions for Use
We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
Components
We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human NPHP3 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of NPHP3 sirna 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.
Related Product Information for
NPHP3 sirna
siRNA to inhibit NPHP3 expression using RNA interference
Applications Tested/Suitable for NPHP3 sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for NPHP3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_694972.3
[Other Products]
NCBI GenBank Nucleotide #
NM_153240.4
[Other Products]
UniProt Primary Accession #
Q7Z494
[Other Products]
UniProt Secondary Accession #
Q5JPE3; Q5JPE6; Q68D99; Q6NVH3; Q7Z492; Q7Z493; Q8N9R2; Q8NCM5; Q96N70; Q96NK2[Other Products]
UniProt Related Accession #
Q7Z494[Other Products]
Molecular Weight
15,798 Da
NCBI Official Full Name
nephrocystin-3
NCBI Official Synonym Full Names
nephronophthisis 3 (adolescent)
NCBI Official Symbol
NPHP3 [Similar Products]
NCBI Official Synonym Symbols
MKS7; NPH3; RHPD; RHPD1; SLSN3; CFAP31
[Similar Products]
NCBI Protein Information
nephrocystin-3
UniProt Protein Name
Nephrocystin-3
Protein Family
Nephrocystin
UniProt Gene Name
NPHP3 [Similar Products]
UniProt Synonym Gene Names
KIAA2000 [Similar Products]
UniProt Entry Name
NPHP3_HUMAN
NCBI Summary for NPHP3
This gene encodes a protein containing a coiled-coil (CC) domain, a tubulin-tyrosine ligase (TTL) domain, and a tetratrico peptide repeat (TPR) domain. The encoded protein interacts with nephrocystin, it is required for normal ciliary development, and it functions in renal tubular development. Mutations in this gene are associated with nephronophthisis type 3, and also with renal-hepatic-pancreatic dysplasia, and Meckel syndrome type 7. Naturally occurring read-through transcripts exist between this gene and the downstream ACAD11 (acyl-CoA dehydrogenase family, member 11) gene. [provided by RefSeq, Feb 2011]
UniProt Comments for NPHP3
NPHP3: Required for normal ciliary development and function. Inhibits disheveled-1-induced canonical Wnt-signaling activity and may also play a role in the control of non-canonical Wnt signaling which regulates planar cell polarity. Probably acts as a molecular switch between different Wnt signaling pathways. Required for proper convergent extension cell movements. Defects in NPHP3 are the cause of nephronophthisis type 3 (NPHP3); also known as adolescent nephronophthisis. NPHP3 is a autosomal recessive disorder resulting in end-stage renal disease. It is characterized by polyuria, polydipsia, anemia. Onset of terminal renal failure occurr significantly later (median age, 19 years) than in juvenile nephronophthisis. Renal pathology is characterized by alterations of tubular basement membranes, tubular atrophy and dilation, sclerosing tubulointerstitial nephropathy, and renal cyst development predominantly at the corticomedullary junction. Defects in NPHP3 are a cause of renal-hepatic-pancreatic dysplasia (RHPD). RHPD is an autosomal recessive disorder with variable expression, and patients surviving the neonatal period progress to renal and hepatic failure which can be treated successfully with combined liver-kidney transplantation. Defects in NPHP3 are the cause of Meckel syndrome type 7 (MKS7). It is a form of Meckel syndrome, an autosomal recessive disorder. It is characterized by a combination of renal cysts and variably associated features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia and cysts, and polydactyly. 7 isoforms of the human protein are produced by alternative splicing.
Chromosomal Location of Human Ortholog: 3q22.1
Cellular Component: cytosol; cilium
Molecular Function: protein binding
Biological Process: Wnt receptor signaling pathway; convergent extension involved in gastrulation; organelle organization and biogenesis; photoreceptor cell maintenance; heart looping; determination of left/right symmetry; kidney development; maintenance of organ identity; lung development
Disease: Meckel Syndrome, Type 7; Renal-hepatic-pancreatic Dysplasia 1; Nephronophthisis 3
Research Articles on NPHP3
1. a rare neonatal ciliopathy presentation of NPHP3 mutations leading to severe multiorgan failure in two siblings.
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.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
