CTNS antibody (AA 231-330)

Catalog No. ABIN1713755

Product Details anti-CTNS Antibody

Target: CTNS (Cystinosis, Nephropathic (CTNS))

Binding Specificity: AA 231-330

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CTNS antibody is un-conjugated

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

Predicted Reactivity: Human,Mouse,Rat,Rabbit

Purification: Purified by Protein A.

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

Isotype: IgG

Application Details

Application Notes: 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

Handling

Format: Liquid

Concentration: 1 μg/μL

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

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

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

Expiry Date: 12 months

Target Details for CTNS

Target: CTNS (Cystinosis, Nephropathic (CTNS))

Alternative Name: CTNS (CTNS Products)

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

Background:

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.

Gene ID: 1497