α-galactosidase

α-galactosidase Structure
α-galactosidase structure
Common Name α-galactosidase
CAS Number 9025-35-8 Molecular Weight 192.602
Density 1.4±0.1 g/cm3 Boiling Point 324.4±22.0 °C at 760 mmHg
Molecular Formula C9H5ClN2O Melting Point N/A
MSDS N/A Flash Point 150.0±22.3 °C
Symbol GHS07
GHS07
Signal Word Warning

High throughput screening for inhibitors of alpha-galactosidase.

Curr. Chem. Genomics 4 , 67-73, (2011)

Fabry disease is a rare X-linked lysosomal storage disorder caused by a deficiency in α-galactosidase A (GLA), which catalyzes the hydrolysis of terminal α-galactosyl groups from glycosphingolipids, such as globotriaosylceramide (Gb3). Many of the mutations i...

Endothelial nitric oxide synthase uncoupling and microvascular dysfunction in the mesentery of mice deficient in α-galactosidase A.

Am. J. Physiol. Gastrointest. Liver Physiol. 306(2) , G140-6, (2014)

A defect in the gene for the lysosomal enzyme α-galactosidase A (Gla) results in globotriaosylceramide (Gb3) accumulation in Fabry disease and leads to premature death from cardiac and cerebrovascular events. However, gastrointestinal symptoms are often first...

Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome.

PLoS ONE 7(10) , e47805, (2012)

Enzyme replacement therapy (ERT) with alpha-Galactosidase A (aGal A) may cause antibody (AB) formation against aGal A in males with Fabry disease (FD). Anti agalsidase ABs negatively influence globotriaosylceramide (Gb3) reduction. We investigated the impact ...

Cicer α-galactosidase immobilization onto functionalized graphene nanosheets using response surface method and its applications.

Food Chem. 142 , 430-8, (2014)

Cicer α-galactosidase was immobilized onto functionalized graphene with immobilization efficiency of 84% using response surface methodology (Box-Behnken design). The immobilized enzyme had higher thermal stability than the soluble one, attractive for industri...

Fabry's disease--a comprehensive review on pathogenesis, diagnosis and treatment.

J. Pak. Med. Assoc. 64(2) , 189-94, (2014)

Fabry's is a progressive, destructive and life threatening disease which reduces significantly life expectancy of the affected individual. It is a genetic disorder of X-linked inheritance caused by deficiency of lysosomal enzyme alpha-galactosidase A resultin...

Angiokeratomas - When is a few too many?

Int. J. STD AIDS 25(5) , 378-9, (2014)

Many patients have a few scattered angiokeratoma and we reassure them that this it is normal; however, if they are numerous, Fabry disease should be considered and the family history should be checked.

Alpha-galactosidase from Mortierella vinacea. Crystallization and properties.

J. Biol. Chem. 245(4) , 781-6, (1970)

Fabry disease: dose matters.

J. Am. Soc. Nephrol. 25(4) , 653-5, (2014)

The Ca2+-activated K+ channel of intermediate conductance: a molecular target for novel treatments?

Ann. Allergy Asthma Immunol. 112(1) , 71-2, (2014)

This review discusses the Ca2+-activated K+ channels of intermediate conductance (IK channels), and their historical discovery in erythrocytes, their classical biophysical characteristics, physiological function, molecular biology as well as their role as pos...

Red meat allergy in Sweden: association with tick sensitization and B-negative blood groups.

J. Allergy Clin. Immunol. 132(6) , 1431-4, (2013)