CTNS anticorps (AA 231-330)

N° du produit ABIN1713755

Détail du produit anti-CTNS anticorps

Antigène: CTNS (Cystinosis, Nephropathic (CTNS))

Épitope: AA 231-330

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp CTNS est non-conjugé

Application: ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunocytochemistry (ICC)

Homologie: Human,Mouse,Rat,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human CTNS/Cystinosin

Isotype: IgG

Information d'application

Indications d'application: ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: 4 °C,-20 °C

Stockage commentaire: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Date de péremption: 12 months

Détails sur CTNS

Antigène: CTNS (Cystinosis, Nephropathic (CTNS))

Autre désignation: CTNS (CTNS Produits)

Synonymes: anticorps CTNS-LSB, anticorps PQLC4, anticorps AI195360, anticorps AW049661, anticorps zgc:110194, anticorps CTNS, anticorps cystinosin, lysosomal cystine transporter, anticorps cystinosis, nephropathic, anticorps cystinosin, lysosomal cystine transporter L homeolog, anticorps CTNS, anticorps Ctns, anticorps ctns, anticorps ctns.L

Sujet:

Synonyms: CTNS LSB, Cystinosin, cystinosis, nephropathic, PQLC4, CTNS_HUMAN.

Background: Cystinosis is an autosomal recessive disorder resulting from defective lysosomal transport of cystine and present at birth as a failure to thrive, rickets and proximal renal tubular acidosis. The human CTNS gene on chromosome 17p13 encodes the protein Cystinosin, and mutations in CTNS are responsible for nephropathic cystinosis. The CTNS promoter contains an Sp1 binding element. Cystinosin is an integral membrane protein containing 7 transmembrane domains that functions as a H+-driven transporter responsible for cystine export from lysosomes. In humans, Cystinosin is expressed abundantly in pancreas, kidney (mature and fetal), and skeletal muscle. The mouse homolog to CTNS encodes a protein which is expressed in all tissues except skeletal muscle. In the cell, Cystinosin co-localizes with LAMP-2 to lysosomes. A C-terminal GYDQL sorting motif within Cystinosin is critical for lysosomal localization.

ID gène: 1497