BS1094

antibody from Bioworld Technology, Inc
Targeting: DYSF FER1L1, LGMD2B
Provider product page for BS1094
Western blot
Immunocytochemistry

Antibody data

Product number
BS1094 - Provider product page
Provider
Bioworld Technology, Inc
Proper citation
Bioworld Technology Cat#BS1094, RRID:AB_1662697
Product name
Dysferlin (P2013) polyclonal antibody
Antibody type
Polyclonal
Antigen
Synthetic peptide, corresponding to amino acids 1981-2030 of Human Dysferlin.
Description
Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin, a protein with a molecular mass of approximately 230 kDa, localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also nvolved in the clinical phenotype. The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
DYSF protein structure - BS1094 shown in red.