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Anti-Dysferlin Antibody

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产品名称: Anti-Dysferlin Antibody
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Anti-Dysferlin Antibody


Anti-Dysferlin Antibody  的详细介绍
Name: Anti-Dysferlin Antibody
See all Dysferlin primary antibodies
Description: Rabbit polyclonal antibody to Dysferlin
Specificity: The antibody detects endogenous levels of total Dysferlin protein.
Applications: WB
Reactivity: Human, Mouse
Immunogen: Synthesized peptide derived from human Dysferlin.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration: 1.0mg / ml
Formulation: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage: Store at -20˚C
Function: Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress (By similarity).
Tissue Specificity: Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Ubiquitous. Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas.
Involvement in Disease: Limb-girdle muscular dystrophy 2B: An autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.

Miyoshi muscular dystrophy 1: A late-onset muscular dystrophy involving the distal lower limb musculature. It is characterized by weakness that initially affects the gastrocnemius muscle during early *****hood.

Distal myopathy with anterior tibial onset: Onset of the disorder is between 14 and 28 years of age and the anterior tibial muscles are the first muscle group to be involved. Inheritance is autosomal recessive.
Sequence Similarities: Belongs to the ferlin family.
Cellular Location: Cell membrane > Sarcolemma. Cytoplasmic vesicle membrane. Cell membrane.

Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites (By similarity).
Database Links:
  • Entrez Gene: 8291 Human
  • Entrez Gene: 26903 Mouse
  • Omim: 603009 Human
  • SwissProt: O75923 Human
  • SwissProt: Q9ESD7 Mouse
  • Unigene: 252180 Human
  • Unigene: 220982 Mouse
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    Synonyms:
  • DMAT Antibody
  • DYSF Antibody
  • Dysferlin Antibody
  • Dysferlin limb girdle muscular dystrophy 2B Antibody
  • Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) Antibody
  • DYSF_HUMAN Antibody
  • Dystrophy associated fer 1 like 1 Antibody
  • Dystrophy associated fer 1 like protein Antibody
  • Dystrophy associated fer1 like 1 Antibody
  • Dystrophy associated fer1 like protein Antibody
  • Dystrophy-associated fer-1-like protein Antibody
  • Fer 1 like protein 1 Antibody
  • Fer-1-like protein 1 Antibody
  • Fer1 like protein 1 Antibody
  • FER1L1 Antibody
  • FLJ00175 Antibody
  • FLJ90168 Antibody
  • LGMD 2B Antibody
  • LGMD2B Antibody
  • Limb girdle muscular dystrophy 2B Antibody
  • Limb girdle muscular dystrophy 2B (autosomal recessive) Antibody
  • Miyoshi myopathy Antibody
  • MM Antibody
  • MMD1 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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