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Anti-SEPN1 Antibody

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产品名称: Anti-SEPN1 Antibody
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Anti-SEPN1 Antibody


Anti-SEPN1 Antibody  的详细介绍
Name: Anti-SEPN1 Antibody
See all SEPN1 primary antibodies
Description: Rabbit polyclonal antibody to SEPN1
Specificity: The antibody detects endogenous levels of total SEPN1 protein.
Applications: IHC
Reactivity: Human
Immunogen: Synthetic peptide corresponding to a region derived from internal residues of human selenoprotein N, 1
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Antigen affinity purification.
Concentration: 0.5mg / ml
Formulation: Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage: Store at -20?C
Function: Isoform 2: Plays an important role in cell protection against oxidative stress and in the regulation of redox-related calcium homeostasis. Regulates the calcium level of the ER by protecting the calcium pump ATP2A2 against the oxidoreductase ERO1A-mediated oxidative damage. Within the ER, ERO1A activity increases the concentration of H(2)O(2), which attacks the luminal thiols in ATP2A2 and thus leads to cysteinyl sulfenic acid formation (-SOH) and SEPN1 reduces the SOH back to free thiol (-SH), thus restoring ATP2A2 activity (PubMed:25452428). Acts as a modulator of ryanodine receptor (RyR) activity: protects RyR from oxidation due to increased oxidative stress, or directly controls the RyR redox state, regulating the RyR-mediated calcium mobilization required for normal muscle development and differentiation (PubMed:19557870, PubMed:18713863).
Tissue Specificity: Isoform 1 and isoform 2 are expressed in skeletal muscle, brain, lung and placenta. Isoform 2 is also expressed in heart, diaphragm and stomach.
Involvement in Disease: Rigid spine muscular dystrophy 1: A neuromuscular disorder characterized by poor axial muscle strength, scoliosis and neck weakness, and a variable degree of spinal rigidity. Early ventilatory insufficiency can lead to death by respiratory failure.

Myopathy, congenital, with fiber-type disproportion: A genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific and can be found in many different myopathic and neuropathic conditions.
Post-Translational Modification: Isoform 2: N-glycosylated.
Cellular Location: Endoplasmic reticulum membrane.
Database Links:
  • Entrez Gene: 57190?Human
  • Omim: 606210?Human
  • SwissProt: Q9NZV5?Human
  • Unigene: 323396?Human
    		•
    Synonyms:
  • CFTD Antibody
  • MDRS1 Antibody
  • RSMD1 Antibody
  • RSS Antibody
  • Selenoprotein N Antibody
  • Selenoprotein N, 1 Antibody
  • SelN Antibody
  • SELN_HUMAN Antibody
  • SEPN1 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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