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anti-CTNS antibody :: Rabbit cystinosis, nephropathic Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS714371 anti-CTNS antibody
Unit / Price
0.05 mL  /  $230 +1 FREE 8GB USB
0.15 mL  /  $500 +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   

cystinosis, nephropathic (CTNS), Polyclonal Antibody

 Also Known As   

Rabbit anti-human cystinosis, nephropathic polyclonal Antibody

 Product Synonym Names    cystinosis; nephropathic; CTNS; CTNS-LSB; PQLC4
 Product Gene Name   

anti-CTNS antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    606272
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Antigen Affinity Purified
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 Immunogen    Human CTNS
 Storage Buffer    PBS with 0.1% 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-134899
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 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-CTNS 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-CTNS antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

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NCBI/Uniprot data below describe general gene information for CTNS. It may not necessarily be applicable to this product.
 NCBI GI #    23273064
 NCBI GeneID    1497
 NCBI Accession #    AAH32850.1 [Other Products]
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 UniProt Secondary Accession #    Q8IZ01; Q9UNK6; D3DTJ5 [Other Products]
 UniProt Related Accession #    O60931 [Other Products]
 Molecular Weight    45,039 Da [Similar Products]
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 NCBI Official Full Name    Cystinosis, nephropathic
 NCBI Official Synonym Full Names    cystinosin, lysosomal cystine transporter
 NCBI Official Symbol    CTNS [Similar Products]
 NCBI Official Synonym Symbols   
PQLC4; CTNS-LSB
[Similar Products]
 NCBI Protein Information    cystinosin
 UniProt Protein Name    Cystinosin
 Protein Family    Cystinosin
 UniProt Gene Name    CTNS [Similar Products]
 UniProt Entry Name    CTNS_HUMAN
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 NCBI Summary for CTNS    This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane. Mutations in this gene cause cystinosis, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2009]
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 UniProt Comments for CTNS    CTNS: Thought to transport cystine out of lysosomes. Defects in CTNS are the cause of cystinosis nephropathic type (CTNS). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. The classical nephropathic form has onset in the first year of life and is characterized by a polyuro-polydipsic syndrome, marked height-weight growth delay, generalized impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, renal failure, ocular symptoms and other systemic complications. Defects in CTNS are the cause of cystinosis adult non- nephropathic type (CTNSANN). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Cystinosis adult non-nephropathic type is characterized by ocular features and a benigne course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Defects in CTNS are the cause of cystinosis late-onset juvenile or adolescent nephropathic type (CTNSJAN). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Late-onset juvenile or adolescent nephropathic cystinosis manifests itself first at age 10 to 12 years with proteinuria due to glomerular damage rather than with the manifestations of tubular damage that occur first in infantile cystinosis. There is no excess amino aciduria and stature is normal. Photophobia, late development of pigmentary retinopathy, and chronic headaches are features. Belongs to the cystinosin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 17p13

Cellular Component: intermediate filament cytoskeleton; intracellular membrane-bound organelle; lysosome; lysosomal membrane; late endosome; early endosome; melanosome; plasma membrane; integral to membrane

Molecular Function: L-cystine transmembrane transporter activity

Biological Process: ATP metabolic process; grooming behavior; amino acid metabolic process; melanin biosynthetic process; lens development in camera-type eye; glutathione metabolic process; long-term memory; L-cystine transport; visual learning; brain development; cognition; adult walking behavior

Disease: Cystinosis, Adult Nonnephropathic; Cystinosis, Late-onset Juvenile Or Adolescent Nephropathic Type; Cystinosis, Nephropathic
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 Research Articles on CTNS    1. The present data exhibit a fundament for molecular carrier detection and prenatal diagnosis of a relatively large percentage of Iranian patients suffering from NC
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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-CTNS antibodyPathways associated with anti-CTNS antibody
 Reference Product  PubMed Publications
 SHPK antibody  >5 publications with CTNS and SHPK
 TRPV1 antibody  >2 publications with CTNS and TRPV1
 Products by Pathway  Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
Diseases associated with anti-CTNS antibodyOrgans/Tissues associated with anti-CTNS antibody
 Disease Name  Pubmed Publications
 Cystinosis Antibodies  >1267 publications with CTNS and Cystinosis
 Male Urogenital Diseases Antibodies  >472 publications with CTNS and Male Urogenital Diseases
 Nervous System Diseases Antibodies  >175 publications with CTNS and Nervous System Diseases
 Endocrine System Diseases Antibodies  >117 publications with CTNS and Endocrine System Diseases
 Liver Diseases Antibodies  >51 publications with CTNS and Liver Diseases
 Growth Disorders Antibodies  >36 publications with CTNS and Growth Disorders
 Atrophy Antibodies  >27 publications with CTNS and Atrophy
 Bone Diseases, Developmental Antibodies  >26 publications with CTNS and Bone Diseases, Developmental
 Heart Diseases Antibodies  >24 publications with CTNS and Heart Diseases
 Autoimmune Diseases Antibodies  >22 publications with CTNS and Autoimmune Diseases
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >488 publications with CTNS and Kidney
 Blood Antibodies  >290 publications with CTNS and Blood
 Eye Antibodies  >185 publications with CTNS and Eye
 Bone Antibodies  >97 publications with CTNS and Bone
 Skin Antibodies  >66 publications with CTNS and Skin
 Liver Antibodies  >65 publications with CTNS and Liver
 Brain Antibodies  >64 publications with CTNS and Brain
 Bone Marrow Antibodies  >54 publications with CTNS and Bone Marrow
 Muscle Antibodies  >52 publications with CTNS and Muscle
 Heart Antibodies  >31 publications with CTNS and Heart
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