AChE Q, Polyclonal Antibody

Acetylcholinesterase collagenic tail peptide; AChE Q subunit; Acetylcholinesterase-associated collagen

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of AChE Q protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-COLQ antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to AChE Q

Western Blot (WB)

WB (1/500 - 1/1000)

Western blot analysis of AChE Q expression in A549 (A), NS-1 (B), H9C2 (C) whole cell lysates.

33,538 Da

collagen-like tail subunit (single strand of homotrimer) of asymmetric acetylcholinesterase

acetylcholinesterase collagenic tail peptide

Acetylcholinesterase collagenic tail peptide

COLQ_HUMAN

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]

COLQ: Anchors the catalytic subunits of asymmetric AChE to the synaptic basal lamina. Defects in COLQ are the cause of congenital myasthenic syndrome Engel type (CMSE); 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. Belongs to the COLQ family. 8 isoforms of the human protein are produced by alternative splicing.

Protein type: Extracellular matrix

Chromosomal Location of Human Ortholog: 3p25

Cellular Component: extracellular space; collagen; basal lamina; synapse; cell junction

Molecular Function: protein binding

Biological Process: asymmetric protein localization; acetylcholine catabolic process in synaptic cleft

Disease: Endplate Acetylcholinesterase Deficiency

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