glycogen storage

branching enzyme

- moves a block of oligoglucose from one chain and adds it to the growing glycogen as a new branch using and aplpha-1,6 glycosidic link

what does enzyme do muscles lack?

glucose-6-phosphatase

phosphorylation of glucose

glucose-6-pophate

pompe disease

- lysosomal alpha-1,4- glucosidase deficiency accumulation of glycogen in lysosomes, muscle hypertonia, death ,

glycogen

An extensively branched glucose storage polysaccharide found in the liver and muscle of animals; the animal equivalent of starch.

andersen disease

GSD - Type IV - Branching Enzyme glycogen with few branches less soluble liver cirrhosis, portal vein hypertension esophageal varices death

McArdle's disease

Muscle phosphorylase deficiency no lactate exercise induced cramping and pain, red urine

binding of glucagon to receptor

activation of PKA which phosphorylation the B FORM OF PHORPHRYLASE. INCREASE GLYCOGEN BREAKDOWN

when glucagon binds to hepatocytes it activates what

adenylate cyclase which increases cAMP and activate PKA

glycogen polymer of glucose residues linked by

alpha (1-4) and (1,6) glycosidic bonds

the activity of hexokinase in muscles means

any free glucose is immediately phosphorylated and enters the glycolytic pathway

what is the major site of daily glucose consumption

brain

glycogenolysis

breakdown of glycogen to glucose occurs by glycogen phosphorylase

released calcium bind what

calmodulin subunit of PhK and this reduces the activity of glycogen synthase

glycogenin

catalyzes its own glycosolation attaching c-1 or UDP glucose to tryosine residue of the enzyme

Epinephrine

cause glycogen break down

protein phosphatases

dephosphorylate glycogen synthase

acetylcoline

depolarize muscle cells and activation phosphorylase kinase

where does the body obtain glucose

diet or from amino acids and lactate via gluconeogenesis

glycogen storage disease

disorders resulting from defects ingenues encoding enzymes involved in process of glycogen synthesis or breakdown

glycogen synthase phosphorylase kinase

enzyme critical for regulation of the flux of glucose into and out of glycogen as it reciprocally regulates the 2 key enzymes of glycogenolysis and glycogen synthesis

Van Gierke disease

enzyme deficiency : glucose 6 phosphatase. increase glycogen accumulation in liver adrenal tubule cells, hypoglycemia, lactic academia ketosis,

what is a major sources of stored glucose

glycogen of skeletal muscle

PKA phosphorylates what

glycogen synthase

activation by G6P doesn't occur when

glycogen synthase is not pohsoprylated

insulin

increases the activity of glycogen synthase

glucagon

inhibits glycogen synthase

de novo glycogen synthesis

initiated by attachment of glucose residue to protein knows glycogen

phophoprotein phosphatase 1

lets it bind to PP1, and thus stops dephosporylating glycogen synthase

glocose 1 phosphate uridyltransferase

makes UDP glucose

udp glucose under going glycogen synthase

makes glycogen +1 glucose

phk

phophorylase kinase that regulate glycogen synthase activity

what influences activity of glycogen synthase?

phosphorylation of serine residues on subunit proteins. this reduces its activity to UDP glucose

glycogenin

protein with self-glycosylating activity that serves as the primer molecules for initiation of glycogen synthesis

debranching enzyme

removes oligosaccharides from a branch in glycogen or starch. can transfer glucose to new branch and then excise the first glucose in the branch as free glucose

glycogen pho

removes single glucose and have that glucose be phosphorylated. can't remove glucose residues rom branch points in glycogen

glycogen synthase

tetrameric enzyme, different in liver and muscle

glycogen synthase

utilizes UDP glucose as 1 substrate and nonreducing end of glycogen

does epinephrine cause the release of glucose from glycogen?

yes