Complex LIpid Metabolism Flashcards
LIPOLYSIS METABOLIC PATHWAYS
- Hydrolysis of neutral fats
- β-oxidation of fatty acids
Triacylglycerol degradation
catalyzed by triacylglycerol lipase
Predominant hormone in the activation of lipolysis
glucagon
fate of glycerol
(liver) gluconeogenesis
Higher triacylglycerol causes
accumulate fat
Steatosis
accumulation of triacylglycerol, causes fatty liver, producing a decrease of the cytosolic enzymes, which causes death of the cell, *fatty liver is steatosis→ which goes from that to necrosis, which is swelling of the liver, which causes death of the cell, and then connective tissue replaces that tissue and causes cirrhosis
Cirrhosis will then lead to
hepatic failure
Tay-Sachs’ disease
Deficiency: Hexosaminidase A
Stored: Ganglioside GM2
ManifestationMental retardation, blindness, cherry red spot
Gaucher’s disease
Deficiency: b-glucocerebrosidase
Stored: Glucocerebroside
Manifestation: Mental retardation, hepato-splenomagaly, erosion of bones
Fabry’s disease
Deficiency: a-galactosidase
Stored: Ceramide trihexoside
Manifestation: Skin rash, kidney failure, pains in lower limbs
Niemann-Pick’s disease
Deficiency: Sphingomyelinase
Stored: Sphingomyelin
Manifestation: Mental retardation, hepatosplenomagaly
Krabbe’s Globoid leukodystrophy
Deficiency: b-galactocerebrosidase
Stored: Galactocerebroside
Manifestation: Mental retardation, absence of myelin
Metachromatic leukodystrophy
Deficiency: Arylsulfatase A
Stored: Sulfolipid
Manifestation: Mental retardation, metachromasia
Generalized gangliosidosis
Deficiency: GM1 b-galactosidase
Stored: Ganglioside GM1
Manifestation: Mental retardation, hepatomegaly, skeletal involvement
Sandhoff-Jatzkewitz disease
Deficiency: b-hexosamidinase B
Stored: Globoside, Ganglioside GM2
Manifestation: Mental retardation, blindness, death before a year of age
