‼️BIOCHEM-Special Topics Flashcards
Examples of acidic sugars
Glucuronic acid
Iduronic acid
GAG in mast cells
Heparin
All GAGs are attached to proteins to form
Proteoglycans
GAG located at sites of calcification in endochondral bone and are also found in cartilage
Chondroitin sulfate
GAGs that play a role in corneal transparency
Keratan sulfate I and dermatan sulfate
GAG Structural role in sclera
Dermatan sulfate
Heparin binds with factors
IX and XI
GAG that determines the charge selectiveness of renal glomerulus
Heparan sulfate
Synthesis of GAGs occur in which organelles?
ER and golgi apparatus
How are GAGs degraded?
They are phagocytosed and vesicles fuse with lysosomes where they are degraded
Lysosomal degradationof GAGs are accomplished by
Acid hydrolases
Accumulation of GAGs in lysosomes due to deficiency in hydrolases
Mucopolysaccharidoses
All mucopolysaccharidoses are autosomal recessive except for
Hunter’s syndrome (x-linked recessive)
All mucopolysaccharidoses have mental retardation except
Morquio’s syndrome
No CNS involvement
Mucopolysaccharidoseses with deficiency in alpha L iduronidase
Hurler’s and Scheie’s syndrome