... impairs metabolism of medium-
chain (C6–C12) fatty acids.
– The C6–C12 fatty acids and their esters accumulate in tissues to cause toxicity.
– Spillover of C6–C10 acylcarnitine species into the ... possible
problems:
– Hepatic dysfunction leading to decreased bile acid synthesis.
– Severe ileal disease leading to malabsorption of bile salts.
– Obstruction of the biliary tr...
... portion of fructose to be excreted in the urine.
86 USMLE Road Map: Biochemistry
N
CLINICAL
CORRELATION
CLINICAL
CORRELATION
102 USMLE Road Map: Biochemistry
N
5. The answer is A. This patient ... agents.
98 USMLE Road Map: Biochemistry
N
Table 7–1 . Stoichiometry of ATP generation from one glucose molecule.
a
NADH FADH
2
ATP
Cytoplasm
Glucose → glucose 6- phosphate...
... frequently lethal soon after birth.
14 USMLE Road Map: Biochemistry
N
CLINICAL
CORRELATION
CLINICAL
CORRELATION
CLINICAL
CORRELATION
34 USMLE Road Map: Biochemistry
N
V
max
V
i
1
2
V
max
0
0
[S]
Positive
... inhibit the enzyme acetylcholinesterase.
– This hydrolase normally breaks down the neurotransmitter acetylcholine within nerve synapses.
28 USMLE Road Map: Bioche...
... is most likely defective?
A. Base excision repair
B. Mismatch repair
164 USMLE Road Map: Biochemistry
N
2. The tRNAs are 6 5–1 10 nucleotides long and their backbones fold back to
allow for intramolecular ... anticodon is frequently inosine (I)
to provide this flexibility in hydrogen
bonding.
166 USMLE Road Map: Biochemistry
N
A. FMR1 (Fragile X)
B. XP-A (Xeroderma pigment...
... representing affected
persons in this pedigree indicate age of diagnosis.
1 96 USMLE Road Map: Biochemistry
N
I
II
III
67
61 37 51 57
54 44 43 38
4. What type of molecular phenomenon is most likely ... with Tay-Sachs disease exhibit hypotonia (poor muscle tone) and progressive neurologic
symptoms, including blindness and mental retardation.
1 86 USMLE Road Map: Biochemistry...
... form (Figure 3 9 6 A
to 3 9 6 C).
The product of the cro gene, the 66 -amino-acid,
9kDa Cro protein, has a single domain but also binds
the operator DNA more tightly as a dimer (Figure
3 9 6 D). The Cro ... to
ch39.qxd 3/ 16/ 04 11:04 AM Page 380
REGULATION OF GENE EXPRESSION /381
Amino acids
132 – 2 36
COOH
ABCD
Cro
O
R
1O
R
3
COOH
NH
2
NH
2
COOH COOH
NH
2
NH
2
Amino acids
1 – 92
C...