Test & Discussion 2024 BIOCHEMISTRY BY DR.

AMIT JAIN

Q1: Impaired degradation of heparan sulphate and its d)Results in a muscular glycogen storage disorder.
subsequent deposition in lysosomes results in?
Q8:Aliver biopsy is done on a child with hepatomegaly
a)Gaucher’s disease. b)Sanfillipo syndrome and mild fastinghypoglycemia.Hepatocytes show
accumulation of glycogen granules withsingle glucose
c)Pompe disease d)Morquio B syndrome
residues remaining at the branch points near the
Q2: Ketogenic diet may be considered as a best dietary peripheryof the granule. The most likely genetic defect
management in deficiency of the following glucose is in the gene encoding :
transporter
a)α-1,4 phosphorylase b)α-1,4:α-1,4 transferase
a)GLUT 1 b)SGLT-1
c)α-1,6 glucosidase d)lysosomal α-1,4 glucosidase
c)GLUT-4 d)GLUT-5
Q9:: A 7-month-old previously healthy male presents
Q3: Which of the following is not associated with high to the emergencyroom for forceful vomiting 3 times
2,3BPG levels? and going limp 1 hour after his mother fed him
formula and 15 ml of grape juice diluted with water.
a)Hyperthyroidism b)High altitude
The infant went to sleep after hismeal and woke up
c)COPD d)G6PD deficiency anaemia screaming and turned pale and went limp. He did not
have any seizure-like activity or apnea. His mother
Q4: Not a fate of Acetyl coenzyme A
rushed him to the emergency room, where hisinitial
a)Fatty acid synthesis b)Cholesterol synthesis blood sugar was 16 mg/dl. A 12 cc IV push of D25 was
administered and hewas started on D5 ½ normal saline
c)Gluconeogenesis d)Tricarboxylic cycle
at maintenance rate. His blood sugar then increasedto
Q5:Which of the following subunit of pyruvate 200 mg/dl. Upon obtaining a more detailed history, his
dehydrogenase complex and its cofactors is not mother stated that the baby had been normal on
correctly matched ? breast feeding. Developmentally the baby was
appropriate for age and he hadhad no significant past
a)E1(pyruvate dehydeogenase) : thiamine
medical history of illnesses. Family history was also
b)E2(dihydrolipoyl transacetylase) : lipoic acid non-contributory. Which of the following enzyme
deficiency can explain above clinical scenario?
c)E3(dihydrolipoyl dehydrogenase) : FAD
a)Fructokinase
d)PDH phosphatase: Coenzyme A
b)Phosphorylase
Q6:Fructose 2,6 bis phosphate as an allosteric modifier
c)Alodolase B
a)Activate gluconeogenesis in fed state
d)Galactose -1-phosphate uridyl transferase.
b)Activate glycolysis in fasting state
Q10:The patient was a 31-year-old male who worked
c)Inhibit Gluconeogenesis in Fed state
in an oil refinery. He was brought to the emergency
d)Inhibit glycolysis in Fasting state department with fever (a temperature of 39.3°C) and
respiratory symptoms. He was hypotensive with a
Q7: Correct of Glucose 6 phosphatase deficiency
blood pressure of 68/40 mm Hg. He reported that he
a)Gluconeogenesis is normal but impaired had been welding in a large container used for the
glycogenolysis storage of sulfur compounds in an open space before
the onset of symptoms. No other chemical compounds
b)Glycogenolysis is normal but impaired
were used and the container was clean at that time,
gluconeogenesis
but there were some unknown fluid residues on the
c)Both gluconeogenesis and glycogenolysis are impaired floor. At the beginning of the welding process, white
fumes with a “rotten egg” odor emanating from the

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

container. The patient immediately felt dizzy and On examination of above x ray, a vitamin deficiency
developed rhinorrhea, teary eyes, nausea, and was made as diagnosis. This vitamin is important for
shortness of breath, chest tightness and cough. These the following post translational modification?
symptoms increased over the following hours followed
a)Glycosylation of hydroxylysine
by hemoptysis. The primary cause of above case can be
explained by inhibition of the following enzyme ? b)Hydroxylation of lysine
a)Cytochrome oxidase c)Carboxylation of glutamate
b)Aconitase d)Methylation of arginine
c)ATP-ADP translocase Q14: Which of the following is not a complication of
therapeutic high doses of niacin?
d)Succinate dehydrogenase
a)Hyperglycemia
Q11:Which of the following enzyme is active when
insulin glucagonratio is low? b)Maculopathies
a)Glucokinase c)Acute fulminant hepatic failure
b)Glycogen phosphorylase d)Hypertriglyceridemia
c)HMG coA Reductase Q15: NADPH is not utilized in
d)Acetyl coenzyme A carboxylase a)Bactericidal activity in neutrophils
Q12:A man presents to the ER with an b)Biosynthesis of cholesterol
levatedtemperature, sweats, and increased rate
c)Oxidative decarboxylation of pyruvate
ofbreathing. He had been spraying insecticideand
accidentally inhaled some of the poison. Using the d)Reduction of oxidized glutathione
insecticide on cultured cells, it wasdemonstrated that
Q16:Not a correct combination of nutritional deficiency
the rate of oxygen consumptionby the cells was much
and cutaneous manifestation
greater than in theabsence of the compound. This drug
is actingmost like which one of the following? a)Zinc: acrodermatitis enetropathica
a)Dintrophenol b)Cyanide b)Copper: pili torti
c)Carbon monoxide d)Rotenone c)Vitamin A: phrynoderma
Q13: d)Niacin: perifollicular haemorrhage
Q17:From a Lineweaver-Burk plot, the Km and Vmax of
HMGcoA reductase,rate-limitingenzyme were
calculated to be 4x10-3 M and 8 x 102 mmol/h,
respectively. If the given experiment is repeated in the
presence of simvastatin, which of the following values
would be obtained?
Km Vmax
a) 4 × 10-3 3 × 102
b)2 × 10-3 1 × 102
c)4 × 10-3 9 × 102
d)8 × 10-3 8 × 102

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

Q18:A 45-year-old man presents with a chief complaint b) Degradation of ganglioside GM2
of intermittent claudicationduring exercise. His family
c) Degradation of glucocerebrosides
history is significant for the presence of
cardiovasculardisease on his father’s side, but not on d) Degradation of sphingomyelin
his mother’s side. Physical exam revealsxanthelasmas
Q22: Which of the following disorder and underlying
and bilateralAchilles’ tendon xanthomas. A plasma
cause is incorrectly matched?
lipid profile revealscholesterol level 340 mg/dL, with a
high LDL/HDL ratio. a) Zellweger syndrome :absent peroxisomes
The clinical findings noted in this patient are most b) Tangiers disease : deficient ABCA1
likely caused by deficient production of
c) Abetalipoproteinemia: deficientmicrosomal
a) lethicin cholesterol acyltransferase triglyceride transfer protein
b) apoB-100 receptors d) Refsum disease: defective omega oxidation
c) fatty acyl-CoA synthetase Q23: Predominant ketone present in blood of a patient
presented with diabetic ketoacidosis, which doesn’t
d) VLDL from LDL
answer Rothera’s test?
Q19: A child is diagnosed with beta oxidation defects
a)Acetoacetate b)Beta hydroxy butyrate
caused by congenital deficiency of medium-chain acyl-
CoA dehydrogenase activity. Which of the following is c)Acetone d)All of the above
diagnostic in this case
Q24: Ketones are not utilized as source of energy, by
a) lactic acidosis b) Ketoacidosis
1)RBC 2)Brain
c) Hyperglycemia d) Dicarboxylic acidosis.
3)Liver 4)Heart
Q20:45-year-old woman with a history of bleeding and
a)1,2 b)1,3
pancytopenia nowpresents with leg pain. She describes
a deep, dull pain of increasing severitythat required c)3,4 d)2,4
pain medication. Computed tomography
Q25: Enzyme deficiency associated with cataract
examinationreveals erosion and thinning of the
formation
femoral head. A bone marrow biopsyis performed to
confirm a diagnosis of Gaucher disease. What 1)Aldose reductase
materialwould be found abnormally accumulating in
2)Galactose 1 phosphate uridyl transferase
the lysosomes of her cells?
3)Sorbitol dehydrogenase
a)Mucopolysaccharide b)Ganglioside
4)Lecithin cholesterol acyl transferase
c)Ceramide d)Cerebroside
a)1,2 b)1,3
Q21:A 17-month-old female infant suspected of a
lysosomal storage disorder onphysical examination c)2,3 d)3,4
reveals a distressedinfant who does not verbalize. Her
Q26: Not a cause of fatty liver
abdomen is tender and enlargement ofboth spleen and
liver are present. Opthalmoscopic examination fails a)Uncontrolled diabetes
toreveal cherry-red spots.A histiocyte in the bone
b)Alcoholism
marrow has a "soap-bubble" appearance. A defect in
which of the following wasmost likely present in this c)Diet rich in Essential fatty acids
infant?
d)Deficiency of lipotropic factors
a) Golgi-associated phosphate transfer to mannose

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

Q27:An otherwise healthy 61-year-old male presented elevated serum ammonia. To discriminate the fact that
with stable angina without traditional risk factors for the hyperammonemia is indeed due to ASS and not
coronary artery atherosclerosis. Lack of traditional risk due to a deficiency in other urea cycle enzymes, one
factors was the impetus for a more extensive workup, can assay for the serum concentration of citrulline and
including specific lipoproteinsincluding Lp(a). The urinary orotic acid concentration.
effect of Lp(a) can partially be explained by its
Which of the following would best describe the
inhibition of
expected findings?
a)Plasmin b)Plasminogen
a) Both citrulline and orotate levels will be
c)Fibrinogen d)Homocysteine
low.
Q28: Rate limiting step of fatty acid synthesis need the
b) Citrulline levels will be absent but orotate
following coenzyme
will be elevated.
a)Biotin b)Vitamin K
c) Citrulline levels will be between
c)Thiamine d)Adenosylcobalmin
100–300 μM and orotate levels will be
Q29: An alcoholic who went on a weekend
bingewithout eating any food was found to elevated.
haveseverehypoglycemia. Hypoglycemia
d) Citrulline levels will be >1000 μM, orotate
occurredbecause the metabolism of ethanol
preventedthe production of blood glucose from will be near normal.
whichone of the following?
Q33:A 25-year-old man has experienced chronic
(A) Glycogen blistering and scarring of his skin when exposed to
sunlight. This man is a smoker and drinks heavily, both
(B) Lactate
of which exacerbate his responses to sunlight. Analysis
(C) Glycerol of his urine and plasma indicates a high accumulation
of complex porphyrins, predominantly uroporphyrin.
(D) Lactate, glycerol, and oxaloacetate and alanine
The symptoms and clinical signs displayed by this
Q30: Synthesis of the following doesn’t require a patient indicate he is suffering from which of the
Kreb’s cycleintermediate? following disorders?
a)Phenylalanine a) acute intermittent porphyria (AIP)
b)Heme b) hereditary coproporphyria (HCP)
c)Glucose c) porphyria cutanea tarda (PCT)
d)Fatty acids d) variegate porphyria
Q31: Which of the following peptide has greater Q34: An infant admitted to the emergency room has
absorbance at 280nm? been found to be suffering from ammonia intoxication,
which was verified by measurement of an elevation of
a)Gln-Leu-Glu-Phe-Thr-Leu-Lys-Asp-Tyr
NH4
b)Tryp-Ser-Gln-Glu-Ala-Tryp-Phe-Tryp-Lys
+ in the serum. Treatment of this infant with arginine
c)Cys-Met-Thr-Tryp-Leu-Gly-Tyr-Ala-Ser results in a reduction serum NH4
d)Glu-Tyr-Phe-Cys-Lys-Tryp-Leu-Val-Thr + and a lessening of the effects of the ammonia
toxicity. The ability of arginine to render this effect
Q32: A deficiency of argininosuccinate synthetase (ASS)
stems from its role in the synthesis of an allosteric
can be suspected in a 24–48-hour-old neonate with

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

activator of the the urea cycle enzyme,
carbamoylphosphate synthetase-I (CPS-I). Which of the
following represents this potent obligate allosteric
effector of CPS-I?
a) argininosuccinate b) bicarbonate ion
c) N-acetylglutamate d) N-acetylcysteine
Q35: Continued consumption of calories in excess of
energy expenditure will eventually lead to obesity, a
current major health problem in the developed
countries. A major contributing factor in obesity is a
disorder in fuel partitioning, as evidenced by a lower
rate of fat oxidation in obese individuals. Which of the
following situations would best explain a reduction in
overall fat metabolism in these individuals?
a) An increase in the hepatic ATP/ adenosine
diphosphate (ADP) ratio increases incorporation of
carbon into fatty acids by causing an inhibition in acetyl-
CoA oxidation in the TCA cycle.
b) Decreased hepatic gluconeogenesis which requires
acetyl-CoA from fatty acid oxidation, thus fat oxidation
is secondarily inhibited.
c) Increased levels of malonyl-CoA occur in these
individuals leading to inhibition of carnitine
palmitoyltransferase I.
d) Insulin-induced decrease in the activity of acetyl-CoA
carboxylase (ACC) causing reduction in fatty acid
synthesis.
Q36:One important function of nitric oxide (NO) is the
induction of vascular smooth muscle relaxation in
response to acetylcholine. The production of NO
requires which amino acid?
a) Arginine b) Asparagine
c) Cysteine d)Lysine
Q37:A 49-year-old man with a rare recessive condition
is at high risk for deep veinthrombosis and stroke and
has had replacement of ectopic lenses. He has a
normalhematocrit and no evidence of megaloblastic
anemia.
Which of the following is not correct regarding this
condition?
b)Amino acid analysis of this patient’s plasma would
most likely reveal anabnormally elevated level of
methionine
c)Cysteine supplements may be required
d)Urine has a characteristic odour of boiled cabbage
Q38:A 56-year-old man with a history of genetic
disease undergoes hipreplacement surgery for
arthritis. During the operation the surgeon notesa dark
pigmentation (ochronosis) in the cartilage. His
ochronotic arthritisis most likely caused by oxidation
and polymerization of excess tissue ?
a) Homogentisic acid b) Orotic acid
c) Methylmalonic acid d) Uric acid
Q39;In an enzyme with a critical Glu residue in the
active site, which of the following amino acid
substitutions would be expected to have the
least effect on enzyme activity?
a) Arg b) Asp
c) Lys d) Ser
Q40: Metabolic disorders not presenting with
hyperuricemia is
a)Lesch nyhan syndrome
b)Hereditary fructose intolerance
c)Von gierkes disease
d)Deficiency of PRPP synthetase
Q41: Amother has brought her 3-month-old baby to
the pediatrician and indicates that the infant is
lethargic and has poor suckling and seems
uninterested in eating. In addition, the mother notes
that the baby’s diapers often smell like burnt sugar.
This infant likely has a defect in which of the following
enzymes?
a) Branched-chain alpha-keto acid dehydrogenase
b) Cystathionine synthase
c) Phenylalanine hydroxylase

a)A mutation in the gene encoding Cystathionine d) Homogentisate oxidase

synthase
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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

Q42: Glycine is not required in synthesis of d) Serotonin to melatonin

a)Heme b)Pyrimidines Q47: Enzyme deficiency
eficiency seen in type 1 Tyrosinemia
c)Purines d)Collagen a)Tyrosine amino transferase.
Q43: A 7-month-old old boy becomes progressively b)4- hydroxyphenyl- pyruvate dioxygenase
lethargic and pale and showsdelayed motor
c)fumarylacetoacetate hydrolase
development. Laboratory evaluation reveals normal
bloodurea nitrogen (BUN), low serum iron, hemoglobin d)homogentisic acid oxidase
5.6 g/dL, and leukopenia.His
ia.His bone marrow shows
Q48:A lack of pantothenic acid would most directly
marked megaloblastosis, which did notrespond to
affect the reaction catalyzed by which
whi one of the
treatment with iron, folic acid, vitamin B12, or
following enzymes?
pyridoxine. Hisurine developed abundant white
precipitate identified as orotic acid. Theunderlying a) Citrate synthase b) Isocitrate dehydrogenase
defect causing the megaloblastic anaemia
aemia in this child
c) Succinate dehydrogenase d) Fumarase
is mostlikely in which of the following pathways?
Q49:
a) Homocysteine metabolism b) Uracil synthesis
c) Urea synthesis d) Heme synthesis
Km Kcat
Q44:A 6-year-oldold boy has had episodic periods during 1 200 nanogram 250
which areas of his skin would develop a rash, which 2 0.4 microgram 250
3 2 microgram 200
would spontaneously resolve in 7to 10 days. Doctor
4 4 microgram 200
told the boy’s parents to give him niacin the next time
this occurred and a high-protein diet, and when they
did, the rash resolved in a day or two. A likely disorder Based on above data , which out of four ,enzyme has
that this child has is which one of the following? highest catalytic efficiency?

a) Cystinuria b) Hartnup disease Q50 :Shown below is a Lineweaver-Burke

Lineweaver plot
displaying the kinetics for an enzyme catalysed
c) Myasthenia gravis d) Alkaptonuria
reaction that was conducted with 800 pmol of enzyme
Q45: Urinalysis of cystinuria patient will not detect in both the absence and presence
presen of a 100 µM
concentration of an inhibitor. Identify the type of
a)Cysteine b)Ornithine
inhibition and calculate the Vmax and Km values in
c)Lysine d)Arginine presence of inhibitor?

Q46:A 3-year-old
old child from Russia, after immigrating
to the
he United States, was found to have developmental
delays and severe mental retardation. Elevated levels
of phenylalanine and phenylpyruvate were found in
the blood. The child was placed on a low low-
phenylalanine diet, but there was no improvement in
the child. Given this information, the child would be
expected to have difficulty in undergoing which one of
the following conversions?
a)Phenylalanine to phenylketones
b)Tyrosine to dopamine
c)Norepinephrine to epinephrine

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

Identify the type of inhibition and calculate the Vmax b)Synthesis of Okazaki fragments
and Km values in presence of inhibitor?
c)Synthesis of RNA primers
nonInhibition Km Vmax
(micromoles) (micromole/min) d)Synthesis of telomeres
A- Non competitive 0.1 0.02 Q56: A 33-year-old man had a screening colonoscopy,
B- Non competitive 10 10
and was diagnosed with a right- sided, mucinous colon
C- Competitive 50 0.02
D- Competitive 10 50 cancer, with no other lesions or polyps seen. The
reason he had a colonoscopy at such an early age is
Q51: Which of the following is a non-functional plasma that his father and paternal uncle had colon cancers
enzyme? diagnosed by age 40. His paternal grandmother had
ovarian and uterine cancers. A likely defect in the
a)Pseudo-cholinestrase patient is a reduction in the ability to carry out which
b)Prothrombin one of the following processes?

c)Creatine kinase a) Removal of thymine dimers from the DNA

d)Lipoprotein lipase b) Inability to remove the base U from DNA

Q52:A double-stranded DNA genome isolated from a c) Loss of DNA ligase activity
bacteria was found to contain 15% thymine. What is d) Inability to correct mismatched bases in newly
the percentageof guanine in this genome? synthesized DNA
a)15 b)25 Q57 : Klenow fragment of prokaryotic DNA polymerase
c)35 d)75 is ?

Q53:Proofreading activity is criticalin determining the a)large fragment of DNA Polymerase I that retains its
accuracy of nuclear DNA replication and thus the base 5'→3' polymerase, 3'→5' exonuclease lacks the 5'→3'
substitution mutation rate in human chromosomes? exonuclease activity of intact DNA polymerase I.

a)A. 3′ to 5′ polymerase activity of DNA polymerase δ
b) 3′ to 5′ exonuclease activity of DNA polymerase γ
c) 3′ to 5′ exonuclease activity of DNA polymerase δ
d) 5′ to 3′ polymerase activity of DNA polymerase III
Q54:Which ofthe following functions is absent in a
patient suffering from xeroderma pigmentosum.
b)large fragment of DNA Polymerase III, that retains its
5'→3' polymerase, 3'→5' exonuclease lacks the 5'→3'
exonuclease activity of intact DNA polymerase I.
c)large fragment of DNA Polymerase I that retains its
5'→3' exonuclease, 3'→5' exonuclease lacks the 5'→3'
polymerase activity of intact DNA polymerase I.
d)is prokaryotic DNA polymerase II .

a)Removal of introns Q58 The process of RNA editing leads to sequence

b)Removal of pyrimidine dimers
c)Repair of wrong bases during replication
d)Repair of mismatched bases during DNA replication
Q55:The enzyme is described as an RNA dependent
DNA polymerase required for chromosome duplication
in the nuclei of rapidly dividing cells, is required in
following step
a)Synthesis of centromeres
changes that are not reflective of the genetic
information contained within the gene for an edited
RNA.Which of the following proteins is an important
example of the result of RNA editing?
a)apo B48 b)calcitonin gene-related peptide
c)ferritin d)glutamate dehydrogenase
Q59:Some viruses, contain a protease that cleaves one
of the eukaryotic initiation factor which identify the

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Test & Discussion 2024 BIOCHEMISTRY BY DR. AMIT JAIN

mRNA cap. Which of the followingfactors is the target of c)Guide RNA

these viral proteases?
d)Base excision repair for double strand breaks.
a)eIF-2 b)eIF-2B
Q66: A young patient with a history of recurring
c)eIF-4A d)eIF-4E attacks of pancreatitis, eruptive xanthomas, normal
cholesterol and increased plasma triglyceride levels
Q60:The bacterial lac operon is controlled by both
(1500 mg/dl) associated with chylomicrons, most likely
glucose and lactose levels. Which of the following
has a deficiency in
conditions would result in the greatest level of
transcription from the lac operon? a)lipoprotein lipase b)LDL receptors
a)both glucose and lactose present c)HMG-CoA reductase d)apoB-48
b)glucose present but no lactose
c)lactose present but no glucose
d)no glucose or lactose present
Q61:An essential requirement for preparation of
recombinant DNA is?
a)Restriction endonuclease b)DNA ligase
c)Polymerase chain reaction d)cDNA
Q62: Incorrect of polymerase chain reaction is
a)exponential amplification of target DNA sample
b)DNA Denaturation primer annealing chain
extension is the basic sequence of reaction
c)“Taq polymerase” is the standard thermostable DNA
polymerase used in PCR applications has no proof
reading activity.
d)Ct (cycle threshold) levels are directly proportional to
the amount of target nucleic acid in the sample
Q63:Post transcriptional modification include all of the
following except
a)5’ capping b)Splicing
c)3’ poly A tailing d)Ubiquitination
Q64:RNAi mediate
a)Gene knock-down b)Gene knock-up
c)Gene knock-in d)Gene knock-out
Q65: Genome editing by CRISPR do not involve
a)Target DNA
b)Cas 9 endonuclease

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