【产品介绍】Dysferlin is a 230 kDa single-pass
type II transmembrane protein; it is a homolog to C.
elegans FER1 protein, which mediates fusion of
intracellular vesicles to the sperm plasma
membrane (1). Dysferlin is highly expressed in
skeletal muscle and commonly localized to the muscle
plasma membrane. A mutation that causes muscular
dystrophy weakens calcium dependent binding of the C2
domain of Dysferlin to phospholipid (2). No or
low immunoreactivity to Dysferlin is seen in
Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and
Miyoshi Myopathy (MM).
Recommended Applications
WB, IHC, ICC
Applications and Recommended Dilution Factors WB: 1:1,000 - 10,000
IHC: 1:50 - 100
ICC: 1:200 - 400
Species Reactivity * Human, Mouse *Species cross-reactivity is based on WB analysis. Products Data
Western blot analysis of human control, human LGMD 2B,
mouse wild type, and mouse Dysf-/- skeletal
muscle homogenates using anti-Dysferlin RabMAb
(cat. #5140-1).
The wild type and Dysf-/- mice were purchased from
The Jackson Laboratory. The control strain is
129S1/SvImJ (stock number 002448). The Dysf-/- strain
is 129-Dysftm1Kcam/J. The donating investigator for
the Dysf-/- strain (Nature 423:168-172, 2003) is
Kevin P. Campbell, The University of Iowa. Images
are kindly provided by Dr. Steven A. Moore from
University of Iowa. Immunohistochemical analysis of paraffin-embedded
human muscle tissue using anti-Dysferlin RabMAb
(cat. #5140-1).
Immunofluorescence image of cat. #5140-1 on unfixed
frozen sections from normal control human skeletal muscle.
Images are kindly provided by Dr. Steven A. Moore
from University of Iowa.
Immunofluorescence image of cat. #5140-1 on unfixed
frozen sections of skeletal muscle from a patient with
LGMD 2B.
Images are kindly provided by Dr. Steven A. Moore
from University of Iowa. Immunofluorescence image of cat. #5140-1 on unfixed
frozen sections from wild type mouse skeletal muscle.
This control mouse strain is 129S1/SvImJ
(stock number 002448) from The Jackson Laboratory. Images are kindly provided by Dr. Steven A. Moore
from University of Iowa. Immunofluorescence image of cat. #5140-1 on unfixed
frozen sections from Dysf-/- mouse skeletal muscle.
This Dysf-/- strain is 129-Dysftm1Kcam/J from The
Jackson Laboratory. The donating investigator for
the Dysf-/- strain (Nature 423:168-172, 2003) is
Kevin P. Campbell, The University of Iowa. Images are kindly provided by Dr. Steven A. Moore
from University of Iowa. Specificity A synthetic peptide corresponding to residues in
human Dysferlin was used as an immunogen. This antibody was made in collaboration with the
Jain Foundation whose goal is to hasten EVERY avenue
that may lead to the cure for LGMD2B/Miyoshi. |
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