Cance: tay-sachs disease and sandhoff disease both belong to a subset of lysosomal storage disorders classified as gm2 gangliosidoses. These diseases are caused by two different enzyme deficiencies, but both diseases result in an accumulation of gm2 gangliosides in the lysosomes of cells, particularly in neurons. The clinical presentation for both diseases is identical. In general, individuals with either condition begin to develop normally, but then present with progressive weakness, hypotonia, increased startle response, progressive neurodegeneration, spasticity, and blindness; death typically occurs by 4 years of age. Tay-sachs disease is an autosomal recessive condition resulting from two mutations in the hexa gene on 15q23-24, which encodes for the alpha subunit of hexosaminidase. Individuals with tay-sachs disease have a deficiency in hexosaminidase a, though those that have higher residual enzyme activity may have a milder clinical presentation with a later age of onset. viagra without prescription The acute infantile form typically presents with progressive motor deterioration beginning at 3 to 6 months of age and death typically occurs by 4 years of age. viagra for daily use bph The juvenile or subacute form often presents with ataxia and incoordination between 2 and 10 years of age with progressive worsening of symptoms and death typically 2 to 4 years later. viagra no prescription usa The chronic or adult-onset subtype may present with motor weakness or psychiatric manifestations in adulthood. generic viagra shipped overnight The carrier frequency of tay-sachs disease is increased in the ashkenazi jewish population (1/31). There are three null alleles with increased frequency in the ashkenazi jewish population that in either homozygous or compound heterozygous state are known to be associated with classical tay-sachs disease (1278instatc, g269s, ivs12+1g->c). viagra or viagra harder The g269s allele results in a higher residual enzyme activity and in either a homozygous or compound heterozygous state results in the adult-onset form of tay-sachs disease. cheap viagra online In addition, ivs9+1g->a and 7. chinese herbal viagra safe 6 kbdel 5'utr-ivs+1 are mutations that are over-represented in individuals of celtic or french canadian ancestry, respectively.. viagra online without prescription ambien cheap viagra generic link online

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