GLYCOGEN STORAGE DISEASES
NAME ENZYME ORGANS CHARACTERESTIC
DEFECTS INVOLVED FEATURES
Von-Gierke’s Glucose-6- Liver,kidney, Glycogen accumulate in
disease phosphatase intestine hepatocyte and renal cells.
Lactic academia
Hyperlipidemia
Ketosis
Gouty arthritis
Pompe’s Lysosomal All organs Glycogen accumulates in
disease ἁ1,4 lysosomes in almost all the
glucosidase tissues, heart is mostly
involved
Nervous system is also
effected
Death occurs at an early age
due to heart failure
Cori’s disease Amylo ἁ 1,6 Liver, muscle, Branched chain glycogen
glucosidase heart, accumulates
leukocytes Liver enlarged
Anderson’s Glycosyl 4,6 Most tissues Glycogen with only few
NAME ENZYME ORGANS CHARACTERESTIC
DEFECTS INVOLVED FEATURES
Von-Gierke’s Glucose-6- Liver,kidney, Glycogen accumulate in
disease phosphatase intestine hepatocyte and renal cells.
Lactic academia
Hyperlipidemia
Ketosis
Gouty arthritis
Pompe’s Lysosomal All organs Glycogen accumulates in
disease ἁ1,4 lysosomes in almost all the
glucosidase tissues, heart is mostly
involved
Nervous system is also
effected
Death occurs at an early age
due to heart failure
Cori’s disease Amylo ἁ 1,6 Liver, muscle, Branched chain glycogen
glucosidase heart, accumulates
leukocytes Liver enlarged
Anderson’s Glycosyl 4,6 Most tissues Glycogen with only few
