Overview

  • Product name

  • Description

    Rabbit polyclonal to CTNS
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow
  • Immunogen

    Recombinant fragment corresponding to Human CTNS aa 282-336.
    Sequence:

    FPQAYMNFYYKSTEGWSIGNVLLDFTGGSFSLLQMFLQSYNNDQWTLIFG DPTKF


    Database link: O60931

  • Positive control

    • A431 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab220391 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Function

    Thought to transport cystine out of lysosomes.
  • Tissue specificity

    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).
  • Involvement in disease

    Cystinosis, nephropathic type (CTNS) [MIM:219800]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Cystinosis, adult, non-nephropathic type (CTNSANN) [MIM:219750]: 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 adult non-nephropathic type is characterized by ocular features and a benigne course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Cystinosis, late-onset juvenile or adolescent nephropathic type (CTNSJAN) [MIM:219900]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Sequence similarities

    Belongs to the cystinosin family.
    Contains 2 PQ-loop domains.
  • Cellular localization

    Lysosome membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • ctns antibody
    • CTNS LSB antibody
    • CTNS_HUMAN antibody
    • Cystinosin antibody
    • Cystinosin, lysosomal cystine transporter antibody
    • cystinosis, nephropathic antibody
    • PQLC4 antibody
    see all

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized A431 cells labeling CTNS with ab220391 at 4 μg/ml (green).

References

ab220391 has not yet been referenced specifically in any publications.

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