Lecture 31 Flashcards
(11 cards)
Phosphorylation of ______-A to form _____-B by the catalytic unit of _____ is inhibitory, which decreases the production of glycogen. This process can be reversed by the hormone _____, which activates protein phosphorylase 1 which dephosphorylates _____-B, converting it to the active form _____-A. Think about how this process might be different for Glycogen Phosphorylase.
GLYCOGEN SYNTHASE-A
GLYCOGEN SYNTHASE-B
PKA
INSULIN
GLYCOGEN SYNTHASE-B
GLYCOGEN SYNTHASE-A.
Von Gierke disease (Type Ia Glycogen storage disese) is characterized by a defect in ________, which converts G-6-P to Glucose in the Liver and Kidneys. It can lead to symptoms like Hypoglycemia, enlarged ______, ______ acidosis because of increased rates of glycolysis, and ketosis.
Glucose-6-Phosphatase
Liver
Lactic acidosis
High levels of G-6-P (from Von Gierke Disease) can activate _______, the normally inactive form, via phosphorylation which essentially results in continuous formation of glycogen in both the liver and muscle tissue. High levels of G-6-P and ATP act to inhibit Glycogen ______ in the liver and muscle tissue, which results in decreased production of glucose-1-phosohate. Keep in mind, in normal conditions, in muscle tissue ONLY, ____ and ____ can stimulate Glycogen ______, resulting in an increase in G-1-P for energy production.
Glycogen synthase-B
Glycogen Phosphorylase
Ca++
AMP
Glycogen Phosphorylase
Type Ib Glycogen storage disease is characterized by a deficiency in _________, which transports ________ into the lumen of the ______ for hydrolase activity.
G-6-P TRANSLOCASE,
G-6-P
ER
Type II Glycogen storage disease (Pompe disease), a lysosomal storage disease, is characterized by a deficiency in Alpha ________. This could lead to symptoms like enlarged _______ and _______ failure. It is considered the most severe Glycogen storage disease. Treatment involves enzyme preparation.
ALPHA GLUCOSIDASE
HEART
CARDIORESPIRATORY
Type III glycogen storage disease (Cori disease) is characterized by a deficiency in _______ enzyme and a notable Glycogen structure feature of short ______ on fasting. It leads to less severe but similar symptoms as Type I, including enlarged _____, moderate ______, and acidosis.
DEBRANCHING
OUTER CHAINS on fasting
Liver
Hypoglycemia
Acidosis.
Type IV Glycogen storage disease (Anderson disease) is characterized by a deficiency in ________ enzyme, which leads to a Glycogen structure containing fewer _____ points (amylopectin-like structure.) It can cause cirrhosis and progressive liver failure.
BRANCHING enzyme
BRANCH points.
Type V Glycogen storage disease (McArdle disease) is characterized by a deficiency in muscle _______ ______. This can lead to symptoms like muscle ______ on exercise, but no increase in serum _____ levels. Similar symptoms can be observed in Type VII because of decreased activity of ____________ in muscle.
GLYCOGEN PHOSPHORYLASE
CRAMPS
Lactate
PHOSPHOFRUCTOKINASE
Type VI Glycogen storage disease (Hers disease) is characterized by a deficiency in liver ______ ______. Symptoms are very similar to Type III, including enlarged ______, moderate ______, and mild ______. A related disease is TypeVIII and is a deficiency in liver _____ _____ _____. It should make sense this has essentially the same effect as Type VI, because the enzyme deficient in Type VIII activates the one in Type VI. Type VIII is ____-linked recessive.
GLYCOGEN PHOSPHORYLASE
LIVER
HYPOGLYCEMIA
ACIDOSIS
Glycogen Phosphorylase Kinase
X-linked
Type IX Glycogen storage disease (the present classification of Type VIII) is characterized by a deficiency in liver ______ ______ ______ and is ____-linked recessive. It’s symptoms are the same as Type VI.
GLYCOGEN PHOSPHORYLASE KINASE
X-linked recessive.
Frequent administration of uncooked _____ is used to treat the symptoms of Type1a and 1b glycogen storage diseases
Corn Starch
