GSD Diseases
This disease is caused by a deficiency in Glucose 6-phosphatase and leads to severe fasting hypoglycemia and hepatomegaly
Von Gierke disease
This polysaccharide serves as the primary storage form of glucose in humans and is mainly found in the liver and skeletal muscle
Glycogen
This 6-carbon molecule is the starting substrate for glycolysis and is trapped in the cell by phosphorylation
Glucose
This major component of the bacterial cell wall consists of a glycan backbone cross-linked by short peptides
Peptidoglycan
This enzyme, found in both saliva and pancreatic secretions, begins the breakdown of starch by cleaving alpha-1,4-glycosidic bonds
Amylase
Unlike other GSDs, Pompe disease is classified as this type of "organelle" storage disease because the defect occurs in the acid maltase enzyme
Lysosomal storage disease
This key regulatory enzyme is active when it is dephosphorylated and is responsible for making linear α-1,4-linked polyglucose chains
Glycogen Synthase
This enzyme catalyzes the "committed step" of glycolysis, converting Fructose 6-phosphate to Fructose 1,6-bisphosphate
Phosphofructokinase-1 (PFK-1)
This hair-like appendage is primarily used for adhesion to host cell surfaces and is a critical virulence factor for bacteria like E. coli
pilus (or fimbria)
To be absorbed by the enterocytes of the small intestine, all dietary carbohydrates must be broken down into this specific molecular form
Monosaccharides
While most GSDs involve the liver, this specific type only affects the muscles, leading to cramps and "burgundy-colored" urine after exercise
McArdle disease
This protein serves as the core "primer" to which the first glucose units are attached during the initiation of glycogen synthesis
Glycogenin
These are the three irreversible enzymes of glycolysis that must be bypassed during gluconeogenesis
TRIPLE POINTS!!!!!
Hexokinase/Glucokinase, Phosphofructokinase-1, and Pyruvate Kinase
This extracellular polysaccharide matrix allows bacterial communities to adhere to surfaces like prosthetic valves or catheters, providing protection against antibiotics
Biofilm
These two GLUT transporters have a low Km (high affinity), ensuring a constant uptake of glucose for the brain and red blood cells even during fasting
DAILY DOUBLE!!!!
GLUT1 & GLUT3
This is the name given to the abnormally short-branched glycogen that accumulates in the cells of patients with Cori disease
Limit Dextrin
In skeletal muscles, an increase in these two allosteric activators—one from ATP hydrolysis and one from nerve impulses—triggers glycogen breakdown
DAILY DOUBLE!!!!
AMP & Calcium (Ca2+)
This vitamin is a required cofactor for the enzyme Pyruvate Carboxylase
Biotin (B7)
This toxic component of the Gram-negative outer membrane is released during cell lysis and can lead to septic shock
Lipid A (the toxic part of LPS)
This 31-amino acid peptide is cleaved from proinsulin and serves as a useful clinical marker to measure endogenous insulin production
C-peptide
This rare GSD involves a branching enzyme deficiency, leading to the accumulation of abnormally long, linear glycogen that the body treats as a foreign body, causing early cirrhosis
Andersen disease
This "dual-function" enzyme is required to move three glucose residues to a longer chain and hydrolyze the remaining α-1,6-bond during glycogen breakdown
Debranching Enzyme
This heavy metal inhibits Glyceraldehyde 3-phosphate dehydrogenase by mimicking phosphate, preventing ATP production
Arsenate
These toxins are secreted by both Gram-positive and Gram-negative bacteria and often have an A-B structural subunit
Exotoxins
Unlike glucagon, which focuses on the liver, this hormone stimulates glycogenolysis in both the liver and the skeletal muscle
Epinephrine