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Medical Biochemistry > Introduction to Biopolymers > Flashcards

Introduction to Biopolymers Flashcards

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USMLE® Step 1 flashcards
1
Q

Which monomers make up Sucrose?

A

Glucose + Fructose

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2
Q

Which monomers make up Lactose?

A

Glucose + Galactose

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3
Q

Which Monomers make up Maltose?

A

Glucose + Glucose

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4
Q

Which type of bond is used to link polymers in lactose?

A

Beta-1,4 Glycosidic bond

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5
Q

Which enzyme breaks down the glycosidic bond in Lactose, and is deficient in lactose intolerance?

A

Lactase

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6
Q

What are the two types of starches?

A
  • Amylose

- amylopectin

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7
Q

What process results in formation of an uronic?

A

Oxidation of sugars at non-anomeric carbons

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8
Q

What are the 3 categories of heteropolysaccharides?

A
  1. Mucopolysaccharides (GAGs)
  2. Proteoglycans
  3. Glycoproteins
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9
Q

What two components make up a GAG?

A
  1. Amino Sugar (GlcNAc or GalNAc)

2. Uronic Acid (GlcA or IdoA)

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10
Q

Describe the Pathology of Hurler Syndrome.

A
  • 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
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11
Q

Describe the pathology of Hunter Syndrome?

A
  • 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
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12
Q

Describe the formation of cataracts in diabetics.

A
  • 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).
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13
Q

What role do glycoproteins play in cell activity?

A
  • Play important role in immune defense

- Form the basis of RBC Blood type

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14
Q

What bond types link which biopolymer?

A

Carbohydrates: glycosidic Bond

Nucleotides: Phosphodiester Bond

Amino Acids: Peptide Bond

Fatty Acid: Ester Bond

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15
Q

Describe linoleic acid.

A
  • 18:2 omega-6 fatty Acid

- Bond position at 9 and 12 from C end

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16
Q

Describe linolenic Acid.

A
  • 18:3 omega-3 FA

- Bond positions at 9,12,15

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17
Q

Describe Arachidonic Acid

A
  • 20:4 Omega-6 FA

- Bond positions at 5,8,11,and 14

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18
Q

What is the clinical significance of Arachidonic Acid?

A

It is a precursor for prostanoids and thrombaxanes

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19
Q

What is the farthest position of double bonds in fatty acids that can be inserted in humans?

A

Position 9

20
Q

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
A

C) Autosomal recessive Hurler syndrome

21
Q

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

A) Metabolic acidosis with respiratory compensation

22
Q

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?

A

Xeroderma pigmentosa

23
Q

What are the two most abundant saturated fatty acids in the body?

A

Palmitic(C16) and stearic acid(C18)

24
Q

What are the three most common unsaturated fatty acids in the body?

A

Oleate , linoleic, and Arachidonic acids

25
Q

Is linoleic acid essential or nonessential?

A

Essential

26
Q

Which nucleic acids contain 2 rings?

A

Purines

27
Q

What type of nucleic acid is Cytosine?

A

Pyrimidine

28
Q

What are common neutral fats?

A
  • MAG, DAG, TAG
29
Q

What is saponification?

A

Hydrolysis of neutral FAs by acids/bases

30
Q

What are the main types of nonglycerol complex lipids?

A
  • Sphingolipids
  • Ceramides
  • Sphingomyelins
  • Cerebrosides
  • Galactosylcerebrosides
  • gangliosides
31
Q

What is the pH of most FAs?

A

4.85

32
Q

Describe the pathology of Tay Sachs disease.

A
  • 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
33
Q

Describe the pathology of Fabry’s disease

A
  • 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
34
Q

What important body lipids are terpenes?

A
  • Vitamin A
  • Cholesterol
  • Vitamin E
  • coenzyme Q
35
Q

What are the positively charged AAs?

A

K, H, N

36
Q

Which AAs are polar and uncharged?

A

S, T, C, P, D, and Q

37
Q

High Amino acids are essential?

A

PVTTIMHLL

38
Q

Describe the pathology of Branch chain Ketoaciduria.

A
  • 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”

39
Q

Describe the pathology of Pellagra

A
  • 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
40
Q

What important molecule is made from His?

A

Histamine - Important in inflammatory process

41
Q

What is the major cause of hyperlysinemias?

A

Inability to metabolize Lys

42
Q

What is Arginine important for?

A
  • Synthesis of NO -> vasodilation/constriction

- Urea cycle

43
Q

What is the pH of gastric secretions?

A
  • 0.7
44
Q

What is the pH of cytosol in the cell? Arterial blood?

A

Cytosol: 7.2

Arterial blood: 7.4

45
Q

What is the pH of pancreatic solutions?

A

8.1

Medical Biochemistry (26 decks)

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