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>   首页   >   产品   >   一抗   >   其他   >   COLQ Polyclonal Antibody   

COLQ Polyclonal Antibody

Purified Rabbit Polyclonal Antibody (Pab)

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF, ICC, E
Primary Accession Q9Y215
Reactivity Rat, Pig
Host Rabbit
Clonality Polyclonal
Calculated MW 47766 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human COLQ
Epitope Specificity 301-400/455
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Cell junction; synapse.
SIMILARITY Belongs to the COLQ family. Contains 2 collagen-like domains.
Post-translational modifications The triple-helical tail is stabilized by disulfide bonds at each end.
DISEASE Defects in COLQ are the cause of congenital myasthenic syndrome Engel type (CMSE) [MIM:603034]; also known as end-plate acetylcholinesterase deficiency or congenital myasthenic syndrome type IC (CMS-IC). CMSE is a rare autosomal recessive congenital myasthenic syndrome characterized by onset during childhood, generalized weakness, abnormal fatigability on exertion, refrectoriness to acetylcholinesterase drugs, decremental electromyographic response and morphological abnormalities of the neuromuscular junctions.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This gene encodes the subunit of a collagen-like molecule associated with acetylcholinesterase in skeletal muscle. Each molecule is composed of three identical subunits. Each subunit contains a proline-rich attachment domain (PRAD) that binds an acetylcholinesterase tetramer to anchor the catalytic subunit of the enzyme to the basal lamina. Mutations in this gene are associated with endplate acetylcholinesterase deficiency. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
Additional Information
Gene ID 8292
Other Names Acetylcholinesterase collagenic tail peptide, AChE Q subunit, Acetylcholinesterase-associated collagen, COLQ
Target/Specificity Found at the end plate of skeletal muscle.
Dilution IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,ELISA=1:5000-10000
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Protein Information
Name COLQ
Function Anchors the catalytic subunits of asymmetric AChE to the synaptic basal lamina.
Cellular Location Synapse.
Tissue Location Found at the end plate of skeletal muscle.
Research Areas

For Research Use Only. Not For Use In Diagnostic Procedures.

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