Introduction to Biopolymers Flashcards
Which monomers make up Sucrose?
Glucose + Fructose
Which monomers make up Lactose?
Glucose + Galactose
Which Monomers make up Maltose?
Glucose + Glucose
Which type of bond is used to link polymers in lactose?
Beta-1,4 Glycosidic bond
Which enzyme breaks down the glycosidic bond in Lactose, and is deficient in lactose intolerance?
Lactase
What are the two types of starches?
- Amylose
- amylopectin
What process results in formation of an uronic?
Oxidation of sugars at non-anomeric carbons
What are the 3 categories of heteropolysaccharides?
- Mucopolysaccharides (GAGs)
- Proteoglycans
- Glycoproteins
What two components make up a GAG?
- Amino Sugar (GlcNAc or GalNAc)
2. Uronic Acid (GlcA or IdoA)
Describe the Pathology of Hurler Syndrome.
- Lysosomal Storage Disease
- Genetic origin; autosomal recessive
- Deficiency in a-L-Iduronidase which is responsible for breaking down various GAGs
- Leads to coarsening of facial features, corneal clouding
- More Severe than Hunter Syndrome
Describe the pathology of Hunter Syndrome?
- Lysosomal storage disease leading to build up of GAGs
- Defect in iduronate-2-sulfatase (I2S)
- X-linked recessive
- Early Life: Runny nose, ear infections, hernias
- By 2 – 4 years of age: cognitive delay and unusual facial features: enlarged tongue and tonsils, enlarged abdomen, broad nose with flared nostrils, thick lips and large jaws, large head, enlarged liver and spleen from GAGs storage, umbilical hernias, airway obstructions, thick nasal secretions
- Distinguishing features: Progressive skeletal changes, stiff joints, behavioral problems, hyperactivity
Describe the formation of cataracts in diabetics.
- Aldose Reductase is the enzyme which catalyzes reduction of glucose to sorbitol.
- Hyperlycemia (High BG) -> INC Blood flow in eye -> shearing of vesssels and macula edema
- High Intracellular glucose -> Conversion to sorbitol -> Osmotic damage
- Retina and Lens have little sorbitol dehydrogenase activity which converts sorbitol to fructose (Fructose enters glycolysis via Hexokinase).
What role do glycoproteins play in cell activity?
- Play important role in immune defense
- Form the basis of RBC Blood type
What bond types link which biopolymer?
Carbohydrates: glycosidic Bond
Nucleotides: Phosphodiester Bond
Amino Acids: Peptide Bond
Fatty Acid: Ester Bond
Describe linoleic acid.
- 18:2 omega-6 fatty Acid
- Bond position at 9 and 12 from C end
Describe linolenic Acid.
- 18:3 omega-3 FA
- Bond positions at 9,12,15
Describe Arachidonic Acid
- 20:4 Omega-6 FA
- Bond positions at 5,8,11,and 14
What is the clinical significance of Arachidonic Acid?
It is a precursor for prostanoids and thrombaxanes
What is the farthest position of double bonds in fatty acids that can be inserted in humans?
Position 9
A 3-year-old boy is brought to the doctor because of loss of previously acquired motor skills. Physical examination shows distinctive coarse facial features, a nose with a flattened bridge, an enlarged tongue, and presence of corneal clouding. Which of the following is most likely the diagnosis?
A) fetal alcohol syndrome B) X-linked recessive Hunter syndrome C) Autosomal recessive Hurler syndrome D) Tay Syndrome Sachs disease E) Down syndrome
C) Autosomal recessive Hurler syndrome
A 50-year-old male with alcohol use disorder presents to the emergency department. His medical history includes only alcohol use disorder, and he is not currently taking any medication. On presentation, he complains of nausea, vomiting, and abdominal pain. He says that he has been drinking heavily for several days but over the last day he has been unable to “keep anything down.” He has the following arterial blood gas (ABG) results: pH 7.2, pCO 25, bicarbonate 15 mEq/L. What’s the patient’s acid/base status?
A) Metabolic acidosis with respiratory compensation
B) Metabolic alkalosis with respiratory compensation
C) Respiratory acidosis with respiratory compensation
D) Respiratory acidosis without respiratory compensation
E) Metabolic acidosis without respiratory compensation
A) Metabolic acidosis with respiratory compensation
What disease is autosomal recessive, and characterized by severe skin abnormalities inducing severe sunburns, freckling, blistering, ulceration, etc due to disturbances in the nucleotide excision repair system?
Xeroderma pigmentosa
What are the two most abundant saturated fatty acids in the body?
Palmitic(C16) and stearic acid(C18)
What are the three most common unsaturated fatty acids in the body?
Oleate , linoleic, and Arachidonic acids
Is linoleic acid essential or nonessential?
Essential
Which nucleic acids contain 2 rings?
Purines
What type of nucleic acid is Cytosine?
Pyrimidine
What are common neutral fats?
- MAG, DAG, TAG
What is saponification?
Hydrolysis of neutral FAs by acids/bases
What are the main types of nonglycerol complex lipids?
- Sphingolipids
- Ceramides
- Sphingomyelins
- Cerebrosides
- Galactosylcerebrosides
- gangliosides
What is the pH of most FAs?
4.85
Describe the pathology of Tay Sachs disease.
- rare autosomal recessive Neurodegenerative disorder
- Lysosomal storage disease
- Mutation of HEXA/B gene which code for HEXA enzyme which hydrolyze GM2-gangliosides
- Accumulation of glycolipids in neurons
- Characterized by cherry red spot on macula of eye, blindness, deafness, seizures, paralysis, and stiff muscles.
- Common in Ashkenazi Jews
Describe the pathology of Fabry’s disease
- X-linked recessive disease with Alpha-galactosidase A deficiency
- Accumulation of ceramide trihexoside
- Decreased sweating, Angiokeratoma (bikini rash), Neuropathy, Corneal opacities, and cardiovascular and renal failure
What important body lipids are terpenes?
- Vitamin A
- Cholesterol
- Vitamin E
- coenzyme Q
What are the positively charged AAs?
K, H, N
Which AAs are polar and uncharged?
S, T, C, P, D, and Q
High Amino acids are essential?
PVTTIMHLL
Describe the pathology of Branch chain Ketoaciduria.
- autosomal recessive disease
- Deficiency in branched chain amino acid dehydrogenase
- unable to process branched chain AAs
- Can lead to developmental delays and eventually death.
“Maple syrup urine disease”
Describe the pathology of Pellagra
- Lack of Trp in diet
- Photodermatitis, hair loss, gloss it is, diarrhea, and mental confusion
- Trp is precursor for nicotinamide nucleotides and serotonin
- Nicotinamide is Vit B3
What important molecule is made from His?
Histamine - Important in inflammatory process
What is the major cause of hyperlysinemias?
Inability to metabolize Lys
What is Arginine important for?
- Synthesis of NO -> vasodilation/constriction
- Urea cycle
What is the pH of gastric secretions?
- 0.7
What is the pH of cytosol in the cell? Arterial blood?
Cytosol: 7.2
Arterial blood: 7.4
What is the pH of pancreatic solutions?
8.1
