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anti-RPGRIP1L antibody :: Goat RPGRIP1L Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS421549
Western Blot (WB)
Unit / Price
0.1 mg  /  $300 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

RPGRIP1L, Polyclonal Antibody

 Also Known As   

Goat anti-RPGRIP1L Antibody

 Product Synonym Names    RPGRIP1-like; CORS3; DKFZp686C0668; JBTS7; KIAA1005; MKS5; RPGRIP1-like antibody; CORS3 antibody; DKFZp686C0668 antibody; JBTS7 antibody; KIAA1005 antibody; MKS5 antibody
 Product Gene Name   

anti-RPGRIP1L antibody

[Similar Products]
 Antibody/Peptide Pairs    RPGRIP1L peptide (MBS426359) is used for blocking the activity of RPGRIP1L antibody (MBS421549)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Immunogen Sequence    DKENNKAKRD ILK
 OMIM    216360
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 Clonality    Polyclonal
 Host    Goat
 Species Reactivity    Tested: Human; Expected from sequence similarity: Human, Dog,
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 Purity/Purification    Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
 Form/Format    Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
 Concentration    100ug specific antibody in 200ul (lot specific)
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 Immunogen    Peptide with sequence C-DKENNKAKRDILK, from the internal region of the protein sequence according to NP_056087.2.
 Epitope    Internal region
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 Preparation and Storage    Aliquot and store at -20 degree C. Minimize freezing and thawing.
 ISO Certification    Manufactured in an ISO 9001:2015 Certified Laboratory.
 Other Notes    Small volumes of anti-RPGRIP1L 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.
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 Applications Tested/Suitable for anti-RPGRIP1L antibody   

Peptide ELISA (EIA), Western Blot (WB)

 Application Notes for anti-RPGRIP1L antibody    Peptide ELISA: Antibody detection limit dilution 1: 64000.
Western Blot: Approx 150kDa band observed in Human Brain (Cerebellum) lysates (calculated MW of 151kDa according to NP_056087.2). Recommended concentration: 0.3-1ug/ml.
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 Western Blot (WB) of anti-RPGRIP1L antibody    (0.3ug/ml) staining of Human Brain (Cerebellum) lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.
anti-RPGRIP1L antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for RPGRIP1L. It may not necessarily be applicable to this product.
 NCBI GI #    118442834
 NCBI GeneID    23322
 NCBI Accession #    NP_056087.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_015272.3 [Other Products]
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 UniProt Secondary Accession #    Q9Y2K8; A0PJ88 [Other Products]
 UniProt Related Accession #    Q68CZ1 [Other Products]
 Molecular Weight    142,792 Da
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 NCBI Official Full Name    protein fantom isoform a
 NCBI Official Synonym Full Names    RPGRIP1 like
 NCBI Official Symbol    RPGRIP1L [Similar Products]
 NCBI Official Synonym Symbols   
FTM; MKS5; CORS3; JBTS7; NPHP8; PPP1R134
[Similar Products]
 NCBI Protein Information    protein fantom
 UniProt Protein Name    Protein fantom
 UniProt Synonym Protein Names   
Nephrocystin-8; RPGR-interacting protein 1-like protein; RPGRIP1-like protein
 UniProt Gene Name    RPGRIP1L [Similar Products]
 UniProt Synonym Gene Names    FTM; KIAA1005; NPHP8; RPGRIP1-like protein [Similar Products]
 UniProt Entry Name    FTM_HUMAN
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 NCBI Summary for RPGRIP1L    The protein encoded by this gene can localize to the basal body-centrosome complex or to primary cilia and centrosomes in ciliated cells. The encoded protein has been found to interact with nephrocystin-4. Defects in this gene are a cause of Joubert syndrome type 7 (JBTS7) and Meckel syndrome type 5 (MKS5). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
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 UniProt Comments for RPGRIP1L    RPGRIP1L: Negatively regulates signaling through the G-protein coupled thromboxane A2 receptor (TBXA2R). May be involved in mechanisms like programmed cell death, craniofacial development, patterning of the limbs, and formation of the left-right axis. Involved in the organization of apical junctions in kidney cells together with NPHP1 and NPHP4. Does not seem to be strictly required for ciliogenesis. 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 RPGRIP1L, influence the clinical outcome. Defects in RPGRIP1L are the cause of Joubert syndrome type 7 (JBTS7). JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermis hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. Defects in RPGRIP1L are the cause of Meckel syndrome type 5 (MKS5). MKS is an autosomal recessive disorder 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. Defects in RPGRIP1L are a cause of COACH syndrome (COACHS). It is a disorder characterized by mental retardation, ataxia due to cerebellar hypoplasia, and hepatic fibrosis. Patients present the molar tooth sign, a midbrain- hindbrain malformation pathognomonic for Joubert syndrome and related disorders. Other features, such as coloboma and renal cysts, may be variable. Belongs to the RPGRIP1 family. 2 isoforms of the human protein are produced by alternative splicing.

Chromosomal Location of Human Ortholog: 16q12.2

Cellular Component: axoneme; centrosome; cilium; cytoplasm; cytosol; intercellular junction; tight junction

Molecular Function: protein binding; thromboxane A2 receptor binding

Biological Process: camera-type eye development; cerebellum development; cilium biogenesis; corpus callosum development; determination of left/right symmetry; embryonic forelimb morphogenesis; embryonic hindlimb morphogenesis; establishment and/or maintenance of cell polarity; establishment of planar polarity; in utero embryonic development; kidney development; lateral ventricle development; liver development; negative regulation of G-protein coupled receptor protein signaling pathway; neural tube patterning; nose development; olfactory bulb development; pericardium development; regulation of smoothened signaling pathway

Disease: Coach Syndrome; Joubert Syndrome 1; Joubert Syndrome 7; Meckel Syndrome, Type 5
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 Research Articles on RPGRIP1L    1. KIAA1005 (rs3213758)is associated with single nucleotide polymorphisms in Korean patients, either non-segmental or segmental type.
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 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.
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Products associated with anti-RPGRIP1L antibodyPathways associated with anti-RPGRIP1L antibody
 Reference Product  PubMed Publications
 CEP290 antibody  >22 publications with RPGRIP1L and CEP290
 TMEM67 antibody  >19 publications with RPGRIP1L and TMEM67
 CC2D2A antibody  >16 publications with RPGRIP1L and CC2D2A
 FTO antibody  >15 publications with RPGRIP1L and FTO
 AHI1 antibody  >12 publications with RPGRIP1L and AHI1
 NPHP1 antibody  >11 publications with RPGRIP1L and NPHP1
 NPHP4 antibody  >8 publications with RPGRIP1L and NPHP4
 TMEM216 antibody  >6 publications with RPGRIP1L and TMEM216
 MKS1 antibody  >6 publications with RPGRIP1L and MKS1
 CC2D2B antibody  >1 publications with RPGRIP1L and CC2D2B
 Products by Pathway  Pathway Diagram
 Anchoring Of The Basal Body To The Plasma Membrane Pathway antibodies  Anchoring Of The Basal Body To The Plasma Membrane Pathway Diagram
 Assembly Of The Primary Cilium Pathway antibodies  Assembly Of The Primary Cilium Pathway Diagram
 Hedgehog 'off' State Pathway antibodies  Hedgehog 'off' State Pathway Diagram
 Organelle Biogenesis And Maintenance Pathway antibodies  Organelle Biogenesis And Maintenance Pathway Diagram
 Signal Transduction Pathway antibodies  Signal Transduction Pathway Diagram
 Signaling By Hedgehog Pathway antibodies  Signaling By Hedgehog Pathway Diagram
Diseases associated with anti-RPGRIP1L antibodyOrgans/Tissues associated with anti-RPGRIP1L antibody
 Disease Name  Pubmed Publications
 Congenital Abnormalities Antibodies  >24 publications with RPGRIP1L and Congenital Abnormalities
 Eye Diseases Antibodies  >20 publications with RPGRIP1L and Eye Diseases
 Kidney Diseases Antibodies  >20 publications with RPGRIP1L and Kidney Diseases
 Obesity Antibodies  >15 publications with RPGRIP1L and Obesity
 Cerebellar Diseases Antibodies  >12 publications with RPGRIP1L and Cerebellar Diseases
 Ciliary Motility Disorders Antibodies  >10 publications with RPGRIP1L and Ciliary Motility Disorders
 Retinal Degeneration Antibodies  >7 publications with RPGRIP1L and Retinal Degeneration
 Fibrosis Antibodies  >6 publications with RPGRIP1L and Fibrosis
 Liver Diseases Antibodies  >5 publications with RPGRIP1L and Liver Diseases
 Joubert Syndrome 7 Antibodies  >3 publications with RPGRIP1L and Joubert Syndrome 7
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >23 publications with RPGRIP1L and Kidney
 Brain Antibodies  >21 publications with RPGRIP1L and Brain
 Eye Antibodies  >20 publications with RPGRIP1L and Eye
 Liver Antibodies  >7 publications with RPGRIP1L and Liver
 Connective Tissue Antibodies  >5 publications with RPGRIP1L and Connective Tissue
 Blood Antibodies  >4 publications with RPGRIP1L and Blood
 Bone Antibodies  >3 publications with RPGRIP1L and Bone
 Muscle Antibodies  >2 publications with RPGRIP1L and Muscle
 Embryonic Tissue Antibodies  >2 publications with RPGRIP1L and Embryonic Tissue
 Thymus Antibodies  >1 publications with RPGRIP1L and Thymus
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