WordNet

first in order of importance; "the alpha male in the group of chimpanzees"; "the alpha star in a constellation is the brightest or main star"
the 1st letter of the Greek alphabet
the beginning of a series or sequence; "the Alpha and Omega, the first and the last, the beginning and the end"--Revelations
early testing stage of a software or hardware product; "alpha version"
any high mountain

PrepTutorEJDIC

アルファ(ギリシア語アルファベットの第1字A,α;英語のA,aに相当) / アルファ星(星座の主星)
高山,高峰

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/05 15:18:39」(JST)

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Lysosomal alpha-glucosidase (also called α-1,4-glucosidase^[1]) is an enzyme that in humans is encoded by the GAA gene.^[2] Errors in this gene cause glycogen storage disease type II (Pompe disease).

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.^[2]

References

^ Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. ISBN 978-0470-23396-2.
^ ^a ^b "Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)".

External links

GeneReview/NIH/UW entry on Glycogen Storage Disease Type II (Pompe Disease)

UpToDate Contents

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1. 糖尿病の治療におけるα-グルコシダーゼ阻害剤およびリパーゼ阻害剤 alpha glucosidase inhibitors and lipase inhibitors for treatment of diabetes mellitus
2. 成人の2型糖尿病における血糖の初期マネージメント initial management of blood glucose in adults with type 2 diabetes mellitus
3. 腸壁気腫症 pneumatosis intestinalis
4. 慢性B型肝炎ウイルス感染の試験研究中の治療 investigational treatments of chronic hepatitis b virus infection
5. Assessment of tumor response in patients receiving systemic and nonsurgical locoregional treatment of hepatocellular cancer

English Journal

A cold-adapted and glucose-stimulated type II α-glucosidase from a deep-sea bacterium Pseudoalteromonas sp. K8.

Li W1,2, Xue Y1,2, Li J1,2, Yuan J1,2, Wang X3, Fang W1,2, Fang Z4,5, Xiao Y1,2.
Biotechnology letters.Biotechnol Lett.2015 Nov 13. [Epub ahead of print]
OBJECTIVES: To express and characterize a putative α-glucosidase, Pagl, from Pseudoalteromonas sp. K8 obtained via genome mining approach.RESULTS: Pagl was expressed and purified to homogeneity, with a molecular mass of 60 kDa. It was optimally active at pH 8.5 and 30 °C, and showed cold-adapted
PMID 26564409

Modeling of cooked starch digestion process using recombinant human pancreatic α-amylase and maltase-glucoamylase for in vitro evaluation of α-glucosidase inhibitors.

Cao X1, Zhang C1, Dong Y1, Geng P2, Bai F3, Bai G1.
Carbohydrate research.Carbohydr Res.2015 Sep 23;414:15-21. doi: 10.1016/j.carres.2015.06.007. Epub 2015 Jun 26.
In human, digestion of cooked starch mainly involves breaking down of α-amylase to α-limit dextrins and small linear malto-oligosaccharides, which are in turn hydrolyzed to glucose by the gut mucosal maltase-glucoamylase (MGAM). Human pancreatic α-amylase (HPA), amino- and carboxyl-terminal porti
PMID 26162745

Enzymatic transglycosylation of ginsenoside Rg1 by rice seed α-glucosidase.

Kim MJ1, Kim YH1, Song GS1, Suzuki Y2, Kim MK1.
Bioscience, biotechnology, and biochemistry.Biosci Biotechnol Biochem.2015 Sep 15:1-11. [Epub ahead of print]
Six α-monoglucosyl derivatives of ginsenoside Rg1 (G-Rg1) were synthesized by transglycosylation reaction of rice seed α-glucosidase in the reaction mixture containing maltose as a glucosyl donor and G-Rg1 as an acceptor. Their chemical structures were identified by spectroscopic analysis, and the
PMID 26372017

Japanese Journal

Novel Mutations in the Gene Encoding Acid .ALPHA.-1,4-glucosidase in a Patient with Late-onset Glycogen Storage Disease Type II (Pompe Disease) with Impaired Intelligence

Internal Medicine 50(24), 2987-2991, 2011
NAID 130002062715

Clinical analysis of five infants with glycogen storage disease of the heart-Pompe's disease.

Japanese Heart Journal 27(1), 25-34, 1986
NAID 130000764638

The adult form of acid maltase (alpha-1. 4-glucosidase)deficiency

Ann Neurol 1, 276-280, 1977
NAID 30008831891

「α-1,4-グルコシダーゼ」

Glucosidase, alpha; acid
Identifiers
Symbols	GAA ; LYAG
External IDs	OMIM: 606800 MGI: 95609 HomoloGene: 37268 ChEMBL: 2608 GeneCards: GAA Gene
EC number	3.2.1.20
Gene ontology
Molecular function	• alpha-glucosidase activity; • carbohydrate binding; • maltose alpha-glucosidase activity;
Cellular component	• lysosome; • lysosomal membrane; • membrane; • extracellular vesicular exosome;
Biological process	• maltose metabolic process; • regulation of the force of heart contraction; • diaphragm contraction; • heart morphogenesis; • glycogen catabolic process; • sucrose metabolic process; • glucose metabolic process; • lysosome organization; • locomotory behavior; • tissue development; • vacuolar sequestering; • muscle cell cellular homeostasis; • neuromuscular process controlling posture; • neuromuscular process controlling balance; • cardiac muscle contraction;
	Sources: Amigo / QuickGO
RNA expression pattern
	More reference expression data
Orthologs
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