[qwiz style=”width: auto !important; min-height: auto !important; border-width: 4px !important; border-color: #0099cc !important; ” align=”center”]

[h] Biochemistry Flashcards

[i] Master this session in just 5 minutes.

[q] ………. is a relatively nontoxic substance, is the major carrier of excess nitrogen from tissues.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEdsdXRhbWluZS4=

[q] In the liver, alanine is transaminated by alanine aminotransferase to pyruvate with the amino group being transferred to α-ketoglutarate to form glutamate. Almost all aminotransferase enzymes use ……… as the amino group acceptor.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IGFscGhhLWtldG9nbHV0YXJhdGUu

[q] ………….. is required to mediate the transamination reaction in the liver.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IFB5cmlkb3hhbCBwaG9zcGhhdGUgKFBMUCkgZGVyaXZlZCBmcm9tIHZpdGFtaW4gQjYu[Qq]

[q] Carbamoyl phosphate synthetase I (CPS) require the presence of ………., a molecule formed by NAGS, as this molecule acts as an allosteric activator of CPS.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IE4tYWNldHlsZ2x1dGFtYXRlIChOQUcpLg==

[q] Carbamoyl phosphate then combines with ornithine to form citrulline in a reaction catalyzed by ………. in the mitochondrial matrix.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IG9ybml0aGluZSB0cmFuc2NhcmJhbW95bGFzZSAoT1RDKS4=[Qq]

[q] What is the most likely diagnosis?

8 years old male child presenting with episodes of vomiting, confusion/coma, asterixis and tachypnea + elevated blood glutamine, and decreased blood urea nitrogen (BUN) + ↑ orotic acid in blood and urine?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IE9ybml0aGluZSB0cmFuc2NhcmJhbXlsYXNlIGRlZmljaWVuY3ku[Qq]

[q]  Hyperammonemia cause Hepatic encephalopathy due to ……..?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]

MS4gRGVwbGV0aW9uIG9mIM6xLWtldG9nbHV0YXJhdGUsIGNhdXNpbmcgaW5oaWJpdGlvbiBvZiB0aGUgS3JlYnMgY3ljbGUu

2. Excess ammonia also depletes glutamate, an excitatory neurotransmitter, and causes accumulation of glutamine, resulting in astrocyte swelling and dysfunction.

3. Hyperammonemia increases the conversion of glutamate into glutamine by glutamine synthetase within astrocytes. The resulting increase in glutamine leads to hyperosmolarity and mitochondrial dysfunction, causing astrocytic swelling and impairment.

[q] ……… and ……… Ketogenic amino acids.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IExldWNpbmUgYW5kIEx5c2luZS4=[Qq]

[q] Arginine, histidine, and lysine are (acidic or basic) amino acids.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IGJhc2ljLg==[Qq]

[q] Primary structure of proteins is the sequence of amino acids linked by ……… bonds.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IGNvdmFsZW50IHBlcHRpZGUu

[q] Proteins may also assume a secondary structure, such as the alpha-helix or beta-sheet. ………… are the principal stabilizing force for the secondary structure of proteins.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEh5ZHJvZ2VuIGJvbmRzLg==

[q] The catecholamine contents of the normal human adrenal medulla are approximately 80% epinephrine and 20% as norepinephrine. This epinephrine-heavy ratio is due to the positive effect of ………….. on the expression of the enzyme PNMT, which converts norepinephrine to epinephrine.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IGNvcnRpc29sLg==

[q] What is the most likely diagnosis?

4 years old patient presenting with intellectual disability, growth retardation, seizures, blue eyes, fair complexion, eczema, musty body odor?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IFBoZW55bGtldG9udXJpYSBkdWXCoHRvIOKGkyBwaGVueWxhbGFuaW5lIGh5ZHJveHlsYXNlIG9yIOKGkyB0ZXRyYWh5ZHJvYmlvcHRlcmluIChCSDQpIGNvZmFjdG9yIChhdHlwaWNhbCBvciBtYWxpZ25hbnQgcGhlbnlsa2V0b251cmlhKS4=

[q] ………. is a non-essential amino acid that becomes essential in the setting of phenylketonuria (PKU).

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IFR5cm9zaW5lLg==[Qq]

[q] Patients with malignant phenylketonuria have increased prolactin levels due to ……….?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IERlY3JlYXNlZCBCSDQgY2F1c2VzIGxvd2VyIGxldmVscyBvZiBkb3BhbWluZS4=[Qq]

[q] What is the most likely diagnosis?

3 months old child with his mother presenting with vomiting, poor feeding, urine smells like burnt sugar while changing the diapers?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IE1hcGxlIHN5cnVwIHVyaW5lIGRpc2Vhc2Uu

[q] Some patients with MSUD improve with ………….. treatment, but most still require lifelong dietary restrictions.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IGhpZ2gtZG9zZSB0aGlhbWluZS4=

[q] What is the most likely diagnosis?

3 years old child with his mother presenting with motion restriction, and significant pain, Bluish-black ear cartilage, and sclerae + urine turns black on prolonged exposure to air?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEFsa2FwdG9udXJpYS4=

[q] Alkaptonuria is an autosomal-recessive disorder in which deficiency of homogentisate oxidase blocks the metabolism of phenylalanine and tyrosine at the level of homogentisic acid, thereby preventing the conversion of ………..?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IHR5cm9zaW5lIHRvIGZ1bWFyYXRlLg==

[q] What is the most likely diagnosis?

25 years old patient presenting with tall long extremities, long tapering fingers, pectus exacavatum, downward and inward lens subluxation, with a previous history of MI?

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEhPTU9DWXN0aW51cmlhOg==
CiYjODIxMTsg4oaR4oaRIEhvbW9jeXN0ZWluZSBpbiB1cmluZSwgT3N0ZW9wb3Jvc2lzLCBNYXJmYW5vaWQgaGFiaXR1cywgT2N1bGFyIGNoYW5nZXMgKGRvd253YXJkIGFuZCBpbndhcmQgbGVucyBzdWJsdXhhdGlvbiksIENhcmRpb3Zhc2N1bGFyIGVmZmVjdHMgKHRocm9tYm9zaXMgYW5kIGF0aGVyb3NjbGVyb3NpcyDihpIgc3Ryb2tlIGFuZCBNSSksIGtZcGhvc2lzLCBpbnRlbGxlY3R1YWwgZGlzYWJpbGl0eS4=

[q] ……. becomes an essential amino acid in patients with homocystinuria.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEN5c3RlaW5lLg==

[q] Many patients with Homocystinuria respond dramatically to ……….. supplementation, which improves residual enzymatic activity and reduces plasma homocysteine levels.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IHB5cmlkb3hpbmUgKEI2KS4=

[q] If a patient is instructed to consume 3000 Cal per day, 900 (30%) of which are to be from protein. Because 1 g of protein yields 4 Cal of energy, this patient should consume (900 Cal /4 Cal) = …….. g/day of protein.

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[x][restart]

[/qwiz]