CTNS Antikörper (AA 231-330)

Produktnummer ABIN1713755

Der Kaninchen Polyklonal Anti-CTNS-Antikörper wurde für ELISA, IHC (p), ICC, IF (cc), IF (p) und IHC (fro) validiert. Er ist geeignet, CTNS in Proben von Human zu detektieren.

Kurzübersicht für CTNS Antikörper (AA 231-330) (ABIN1713755)

Target: CTNS (Cystinosis, Nephropathic (CTNS))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CTNS Antikörper ist unkonjugiert

Applikation: 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))

Produktdetails anti-CTNS Antikörper

Bindungsspezifität: AA 231-330

Homologie: Human,Mouse,Rat,Rabbit

Aufreinigung: Purified by Protein A.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu CTNS

Target: CTNS (Cystinosis, Nephropathic (CTNS))

Andere Bezeichnung: CTNS

Hintergrund:

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.

Gen-ID: 1497