蛋白

重组人类乙酰胆碱受体亚单位甲

MBS964358

0.05毫克(大肠杆菌)

190美元

重组蛋白

重组人类乙酰胆碱受体亚单位甲

乙酰胆碱受体亚单位甲

acetylcholine receptor subunit alpha isoform b; Acetylcholine receptor subunit alpha; acetylcholine receptor subunit alpha; cholinergic receptor nicotinic alpha 1 subunit

CHRNA1

ACHRA;CHNRA甲

CHRNA1;CHRNA1;ACHRA;ACHRD;CHRNA;CMS1A;CMS1B;CMS2A;FCCMS;SCCMS;ACHRA;CHNRA

ACHA_HUMAN

E Coli or Yeast or Baculovirus or Mammalian Cell

21-255; Provide the complete extracellular domain.

255

SEHETRLVAKLFKDYSSVVRPVEDHRQVVEVTVGLQLIQ
LINVDEVNQIVTTNVRLKQGDMVDLPRPSCVTLGVPLFS
HLQNEQWVDYNLKWNPDDYGGVKKIHIPSEKIWRPDLVL
YNNADGDFAIVKFTKVLLQYTGHITWTPPAIFKSYCEII
VTHFPFDEQNCSMKLGTWTYDGSVVAINPESDQPDLSNF
MESGEWVIKESRGWKHSVTYSCCPDTPYLDITYHFVMQR
L

Greater than 90% as determined by SDS-PAGE.

Liquid containing glycerol; lyophilization may be available upon request.

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

Transport

After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.

Cloning and sequence analysis of calf cDNA and human genomic DNA encoding alpha-subunit precursor of muscle acetylcholine receptor.Noda M., Furutani Y., Takahashi H., Toyosato M., Tanabe T., Shimizu S., Kikyotani S., Kayano T., Hirose T., Inayama S., Numa S.Nature 305:818-823(1983) The human medulloblastoma cell line TE671 expresses a muscle-like acetylcholine receptor. Cloning of the alpha-subunit cDNA.Schoepfer R., Luther M., Lindstrom J.M.FEBS Lett. 226:235-240(1988) The human muscle nicotinic acetylcholine receptor alpha-subunit exist as two isoforms a novel exon.Beeson D., Morris A., Vincent A., Newsom-Davis J.EMBO J. 9:2101-2106(1990) Cloning of a cDNA coding for the acetylcholine receptor alpha-subunit from a thymoma associated with myasthenia gravis.Gattenloehner S., Brabletz T., Schultz A., Marx A., Mueller-Hermelink H.-K., Kirchner T.Thymus 23:103-113(1994) Complete sequencing and characterization of 21,243 full-length human cDNAs.Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S., Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.Nat. Genet. 36:40-45(2004)

4557457

NP_000070.1

NM_000079.3

P02708

45.1kD

Acetylcholine Binding And Downstream Events Pathway (1268787); Activation Of Nicotinic Acetylcholine Receptors Pathway (1268788); Effects Of Botulinum Toxin Pathway (138028); ErbB2/ErbB3 Signaling Events Pathway (137955); Highly Calcium Permeable Nicotinic Acetylcholine Receptors Pathway (1268790); Highly Calcium Permeable Postsynaptic Nicotinic Acetylcholine Receptors Pathway (1268793); Neuroactive Ligand-receptor Interaction Pathway (83053); Neuroactive Ligand-receptor Interaction Pathway (462); Neuronal System Pathway (1268763); Neurotransmitter Receptor Binding And Downstream Transmission In The Postsynaptic Cell Pathway (1268786)

The muscle acetylcholine receptor consiststs of 5 subunits of 4 different types: 2 alpha subunits and 1 each of the beta, gamma, and delta subunits. This gene encodes an alpha subunit that plays a role in acetlycholine binding/channel gating. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Nov 2012]

nAChRA1: After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. Defects in CHRNA1 are a cause of multiple pterygium syndrome lethal type (MUPSL). Multiple pterygia are found infrequently in children with arthrogryposis and in fetuses with fetal akinesia syndrome. In lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia, and flexion contractures causing severe arthrogryposis and fetal akinesia. Subcutaneous edema can be severe, causing fetal hydrops with cystic hygroma and lung hypoplasia. Oligohydramnios and facial anomalies are frequent. The alpha subunit is the main focus for antibody binding in myasthenia gravis. Myasthenia gravis is characterized by sporadic muscular fatigability and weakness, occurring chiefly in muscles innervated by cranial nerves, and characteristically improved by cholinesterase-inhibiting drugs. Defects in CHRNA1 are a cause of congenital myasthenic syndrome slow-channel type (SCCMS). SCCMS is the most common congenital myasthenic syndrome. Congenital myasthenic syndromes are characterized by muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. SCCMS is caused by kinetic abnormalities of the AChR, resulting in prolonged endplate currents and prolonged AChR channel opening episodes. Defects in CHRNA1 are a cause of congenital myasthenic syndrome fast-channel type (FCCMS). FCCMS is a congenital myasthenic syndrome characterized by kinetic abnormalities of the AChR. In most cases, FCCMS is due to mutations that decrease activity of the AChR by slowing the rate of opening of the receptor channel, speeding the rate of closure of the channel, or decreasing the number of openings of the channel during ACh occupancy. The result is failure to achieve threshold depolarization of the endplate and consequent failure to fire an action potential. Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Alpha- 1/CHRNA1 sub-subfamily. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Membrane protein, multi-pass; Receptor, misc.; Membrane protein, integral; Channel, ligand-gated; Channel, cation. Chromosomal Location of Human Ortholog: 2q31.1. Cellular Component: cell junction; cell surface; neuromuscular junction; nicotinic acetylcholine-gated receptor-channel complex; plasma membrane; postsynaptic membrane. Molecular Function: acetylcholine binding; acetylcholine receptor activity; ion channel activity; nicotinic acetylcholine-activated cation-selective channel activity. Biological Process: generation of action potential; muscle maintenance; musculoskeletal movement; neuromuscular junction development; neuromuscular process; neuromuscular synaptic transmission; regulation of membrane potential; response to nicotine; signal transduction; skeletal muscle contraction; skeletal muscle growth; synaptic transmission; synaptic transmission, cholinergic; transport. Disease: Multiple Pterygium Syndrome, Lethal Type; Myasthenic Syndrome, Congenital, Fast-channel; Myasthenic Syndrome, Congenital, Slow-channel

0.05毫克(大肠杆菌)

190美元

0.2毫克(大肠杆菌)

460

0.5毫克(大肠杆菌)

750

0.05毫克(杆状病毒)

950

0.05 mg (Mammalian-Cell)

1170