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GTX01908

Dysferlin antibody [Ham1/7B6]

Eine Lieferung in diese Region ist nicht möglich.

SKU:
GTX01908
Zusätzliche Namen:
dysferlin , FER1L1 , LGMD2B , LGMDR2 , MMD1
Anwendung:
IHC-P, IHC-Fr
Physischer Zustand:
Liquid
Spezies-Reaktivität:
Human
Aufreinigung:
Unpurified
Lagerbedingungen:
-20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
Hersteller:
Genetex
Host:
Mouse
Reaktivitäten:
Human
Buffer:
Tissue culture supernatant, 0.09% Sodium azide.
Immunogen:
Synthetic peptide containing amino acids 1999-2016 of the human dysferlin molecule.
Klon:
Ham1/7B6
Uniprot:
O75923
Synonyme:
dysferlin;dystrophy-associated fer-1-like 1;Dystrophy-associated fer-1-like protein;fer-1-like family member 1;fer-1-like protein 1;FER1L1;LGMD2B;LGMDR2;limb girdle muscular dystrophy 2B (autosomal recessive);MMD1
Weitere Details:
The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]
Versandbedingungen:
Blue Ice