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Anti-CTNS Antibody

品牌：Antibodies

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規(guī)格：50μl

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Anti-CTNS Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

Name:	Anti-CTNS Antibody See all CTNS primary antibodies
Description:	Rabbit polyclonal antibody to CTNS.
Applications:	WB, IHC, IF
Dilutions:	WB: 1:500 - 1:2000, IHC: 1:50 - 1:200, IF: 1:50 - 1:100.
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant protein of human CTNS.
Protein Length:	367 (400)
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

Function:	Cystine/H(+) symporter thought to transport cystine out of lysosomes. Plays an important role in melanin synthesis, possibly by preventing melanosome acidification and subsequent degradation of tyrosinase TYR.
Tissue Specificity:	Strongly expressed in pancreas, kidney (*** and fetal), skeletal muscle, melanocytes and keratinocytes. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (*** and fetal). Isoform 2 represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions.
Involvement in Disease:	Cystinosis, nephropathic type: 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. Cystinosis, ***, non-nephropathic type: 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 *** non-nephropathic type is characterized by ocular features and a benign course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Cystinosis, late-onset juvenile or adolescent nephropathic type: 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 is an intermediated form, manifesting 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.
Sequence Similarities:	Belongs to the cystinosin family.
Cellular Location:	Lysosome membrane. Melanosome.
Database Links:	Entrez Gene: 1497?Human Entrez Gene: 83429?Mouse Entrez Gene: 287478?Rat Omim: 606272?Human SwissProt: O60931?Human SwissProt: P57757?Mouse Unigene: 187667?Human Unigene: 259852?Mouse
Synonyms:	CTNS Antibody CTNS LSB Antibody CTNS_HUMAN Antibody Cystinosin Antibody Cystinosin, lysosomal cystine transporter Antibody cystinosis, nephropathic Antibody PQLC4 Antibody
Information:	Target information shown above is from the UniProt Consortium.