13916964679
Note: Application as IHC, only suitable for histochemical staining or fluorescence staining of paraffin-embedded sections. Application as ICC/IF, suitable for histochemical or fluorescent staining of frozen sections, as well as chemical and fluorescent staining at the cellular level.
注意：抗体应用为IHC的，抗体只适合于石蜡切片的组化染色或者荧光染色。
抗体应用为IF/ICC的，抗体适合于冰冻切片的组化染色或者荧光染色，以及细胞水平的化学染色和荧光染色。
ABMART实验方案下载
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.
CTNS LSB; Cystinosin; cystinosis, nephropathic; PQLC4; CTNS_HUMAN.
Human,Mouse,Rat,Rabbit
WB
WB 1:500-2000
42kD
1497
O60931
Lysosome membrane; Multi-pass membrane protein.
Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal).
KLH conjugated synthetic peptide derived from human CTNS/Cystinosin
Rabbit
Polyclonal
IgG
affinity purified by Protein A
Liquid
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
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订购专线：4006-123-828
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