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Biochemistry
New set 2023 UWORLD only
| Question | Answer |
|---|---|
| Which two sugars make up sucrose? | Fructose and glucose |
| Which two sugars make up Lactose? | Galactose and glucose |
| Which two sugars make up Maltose? | Glucose and glucose |
| Mnemonic for disaccharide breakdown | Sum From Lactose Guarantees Melting 2 glucoses |
| What is the pathogenesis of Hartnup disease? | Impaired transport of neutral amino acids in the small intestine and PCT. |
| What is a common cause of Hartnup disease? | Niacin deficiency |
| What are the clinical findings of Hartnup disease? | Pellagra-like skin eruptions and cerebellar ataxia |
| Mode of inheritance of Orotic aciduria | Autosomal recessive |
| What is the pathway affected in Orotic aciduria? | De novo Pyrimidine synthesis pathway |
| What is the enzyme deficient in Orotic aciduria? | UMP synthase |
| Deficient UMP synthase. Dx? | Orotic aciduria |
| What can help or improve symptoms of Orotic aciduria? | Uridine supplementation, as uridine is converted to UMP via nucleoside kinases |
| What type of anemia is seen in Orotic Aciduria? | Megaloblastic anemia |
| How does Acute Mesenteric ischemia develop anion gap metabolic acidosis? | ↓ intestinal blood flow ==> anaerobic environment which leads to ↑↑ NADH/NAD+ ratio ----> ↑↑↑ production of lactate and ↓ conversion of Pyruvate into Acetyl-CoA. |
| What amino acid is responsible for transferring Nitrogen to the liver for disposal? | Alanine |
| Once Alanine goes to liver for disposal, it then transforms into what amino acid for later excretion? | Glutamate |
| In the liver, what converts Alanine into Glutamate, in order to be disposed? | α-ketoglutarate |
| Glycogen debrancher deficiency which causes abnormally short outer chains. Dx? | Cori disease |
| What are some clinical findings of Cori disease? | Hypoglycemia, Ketoacidosis, Hepatomegaly, hypotonia/muscle weakness |
| How is NAD+ produced in anaerobic conditions? | NADH transfers electors to Pyruvate to form lactate and regenerate NAD+ |
| What is the role of NAD+ in glycolysis? | Convert G-3-P ----> 1, 3 BPG |
| What does mtDNA resemble? | prokaryotic DNA |
| What electrolyte efflux/influx balance is most responsible for the value of resting potential? | High K+ efflux and some Na+ influx |
| What are the roles of F2,6-BP? | 1. Activates PFK-1 (↑ glycolysis) 2. Inhibits F1,6BP (↓ gluconeogenesis) |
| What enzyme regulates the concentration of F2,6BP? | PFK-2 |
| PFK-2 leads to ---> | ↑↑↑ F2, 6BP levels in response to insulin |
| F2,6-biphosphatase leads to ---> | ↓↓ F2,6BP levels in response to glucagon |
| What type of bonds are formed between 2,3-BPG and HbA? | Ionic bonds btw ß-subunits of deoxygenated HbA and 2,3-BPG |
| ↑↑↑ bonds between 2,3-BPG and Hb leads to: | Facilitating oxygen release in the peripheral tissues |
| Does HbA or HbF have higher affinity to 2,3-BPG binding? | HbF |
| What is the result of 2,3-BPG binding to HbF rather than HbA? | Higher affinity leads to inability to release oxygen to peripheral tissues. |
| Acute Intermittent Porphyria (AIP) attacks lead to accumulation of: | ALA and porphobilinogen (PGB) |
| What enzyme is deficiency in AIP? | PBG deaminase |
| What are the levels of bilirubin seen in Benign Neonatal Hyperbilirubinemia? | ↑↑↑ bilirubin production and enterohepatic circulation ↓↓ Bilirubin conjugation |
Created by:
rakomi
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