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Anti-CLCN7 Antibody

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产品名称: Anti-CLCN7 Antibody
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Anti-CLCN7 Antibody


Anti-CLCN7 Antibody  的详细介绍
Name: Anti-CLCN7 Antibody
See all CLCN7 primary antibodies
Description: Rabbit polyclonal antibody to CLCN7.
Applications: WB, IHC, IF
Dilutions: WB: 1:500 - 1:2000, IHC: 1:50 - 1:200.
Reactivity: Human, Mouse, Rat
Immunogen: Recombinant protein of human CLCN7.
Protein Length: 805
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: Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.
Tissue Specificity: Brain, testis, muscle and kidney.
Involvement in Disease: Osteopetrosis, autosomal recessive 4: A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or *****hood. Recessive osteopetrosis commonly manifests in early infancy with macrocephaly, feeding difficulties, evolving blindness and deafness, bone marrow failure, severe anemia, and hepatosplenomegaly. Deafness and blindness are generally thought to represent effects of pressure on nerves.

Osteopetrosis, autosomal dominant 2: A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or *****hood. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or *****hood. It is characterized by sclerosis, predominantly involving the spine, the pelvis and the skull base.

Osteopetrosis, autosomal recessive 2: A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or *****hood. Recessive osteopetrosis commonly manifests in early infancy with macrocephaly, feeding difficulties, evolving blindness and deafness, bone marrow failure, severe anemia, and hepatosplenomegaly. Deafness and blindness are generally thought to represent effects of pressure on nerves. OPTB2 is characterized by paucity of osteoclasts, suggesting a molecular defect in osteoclast development.
Sequence Similarities: Belongs to the chloride channel (TC 2.A.49) family. ClC-7/CLCN7 subfamily.
Cellular Location: Lysosome membrane.
Database Links:
  • Entrez Gene: 1186?Human
  • Entrez Gene: 26373?Mouse
  • Entrez Gene: 29233?Rat
  • Omim: 602727?Human
  • SwissProt: P51798?Human
  • SwissProt: O70496?Mouse
  • SwissProt: P51799?Rat
  • Unigene: 459649?Human
  • Unigene: 270587?Mouse
  • Unigene: 10338?Rat
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    Synonyms:
  • Chloride channel protein 7 Antibody
  • CLC 7 Antibody
  • ClC-7 Antibody
  • CLC7 Antibody
  • CLCN7 Antibody
  • CLCN7_HUMAN Antibody
  • FLJ26686 Antibody
  • FLJ39644 Antibody
  • FLJ46423 Antibody
  • H(+)/Cl(-) exchange transporter 7 Antibody
  • OPTA2 Antibody
  • OPTB4 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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