产品资料

Anti-CTNS Antibody

如果您对该产品感兴趣的话,可以
产品名称: Anti-CTNS Antibody
产品型号:
产品展商: 其它品牌
产品文档: 无相关文档

简单介绍

Anti-CTNS Antibody


Anti-CTNS Antibody  的详细介绍
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.
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.
    产品留言
    标题
    联系人
    联系电话
    内容
    验证码
    点击换一张
    注：1.可以使用快捷键Alt+S或Ctrl+Enter发送信息!
    2.如有必要,请您留下您的详细联系方式!

    沪公网安备 31011202007337号