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Z - M1 Biochem > M1 Biochem Enzymes - FA Only > Flashcards

M1 Biochem Enzymes - FA Only Flashcards

0
Q

Pyruvate Carboxylase - Function/Pathway/Regulation/Co-Factor/Disorder

A

Function - Converts Pyruvate to Oxaloacetate for
Pathway - Pushes toward Gluconeogenesis - Occurs in Mito.
Regulation - Aerobic Fasting Conditions - Need Glucose (Glucagon)
Co-Factor - ABC - ATP + Biotin + CO2 + Acetyl CoA
Disorder - Avidin (Egg Whites) - Limits Biotin availability - Non functional Pyruvate Carboxylase

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1
Q

Pyruvate DH - Function/Pathway/Regulation/Co-Factor/Disorder

A

Function - Converts Pyruvate to Acetyl CoA
Pathway - TCA Cycle Precursor
Regulation - Acetyl CoA/ATP/NADH Inhibit vs. Ca/ADP Increase + Insulin Stimulates
Co-Factors - 5 - Thiamine + Riboflavin + Niacin + Pantothenate + Lipoic Acid (Not a vitamin)
Deficiency - Thiamine Deficiency - Limits E1 Subunit - BeriBeri + WK
Pyruvate DH Deficiency - Lactic Acidosis + Increase Alanine (ALT) + Neurologic Symptoms

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2
Q

Lactate DH - Function/Pathway/Regulation/Co-Factor

A

Function - Convert Pyruvate to Lactate in anaerobic state
Pathway - Pyruvate Metabolism - Anaerobic State - Major in RBCs Lens + Anaerobic Organs
Regulation - High NADH
Co-Factors - Niacin

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3
Q

Alanine Aminotransferase - Function/Pathway/Regulation/Co-Factor

A

Function - Carriers amino groups to the liver from the muscle (Liver transport for breakdown + ammonia/urea generation)
Pathway - Amino Acid Degradation
Regulation -
Co-Factor - Pyridoxine

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4
Q

Citrate Synthase - Function/Pathway/Regulation

A

Function - Creates Citrate from Acetyl CoA + Oxaloacetate
Pathway - TCA Cycle
Regulation - Inhibited by High Energy (ATP + NADH + Citrate + Succinyl CoA) + Activated by ADP/Ca)

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5
Q

Isocitrate DH - Function/Pathway/Regulation

A

Function - Rate Limiting Step of TCA Cycle - Isocitrate to A-Keto
Pathway - TCA Cycle
Regulation - ATP/NADH Inhibit + ADP Increases

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6
Q

Alpha Ketogluterate DH - Function/Pathway/Regulation/Co-Factor/Disorder

A

Function - Makes Succinyl CoA from A-Keto
Pathway - TCA Cycle
Regulation - Inhibited by NADH + ATP + Succinyl-CoA
Co-Factors - Match PDH - Thiamine + Riboflavin + Niacin + Pantothenate + Lipoic Acid
Disorder - Knocked out in Thiamine Deficiency ( BeriBeri/ WK)

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7
Q

Glycogen Synthase - Function/Pathway/Regulation

A

Function - Rate Limiting Step in Adding UDP-Glucose to glycogen chains (after conversion from Glucose 6P to Glucose 1P to UDP Glucose
Pathway - Glycogenesis
Regulation - Insulin + Glucose 6P activates //// Glucagon + Epi Inhibits

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8
Q

Branching Enzyme - Function/Pathway

A

Function - Transfers groups of at least 6 residues to form a new branch (A-1-6 Link)
Pathway - Glycogenesis

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9
Q

De-Branching Enzyme - 4-Alpha-Glucotransferase - Function/Pathway

A

Function - Moves 3 of the Last 4 Residues to another chain

Pathway - Glycogenolysis

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10
Q

De-Branching Enzyme - Alpha 1-6 Glycosidase - Function/Pathway

A

Function - Removes the Last remaining Glucose 1P off Glycogen branches (straight to glucose)
Pathway - Glycogenolysis

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11
Q

Glycogen Phosphorylase - Function/Pathway/Regulation/Disorder

A

Function - Rate Limiting Step - Removes the glucose-1P’s off glycogen during glycogenolysis
Pathway - Glycogenolysis
Regulation - Activated by Epi/Glucagon + AMP ///// Inhibited by Insulin + ATP + Glucose 6-Phosphate
Disorder - McArdel (Muscle) + Her’s (Liver) - Exercise intolerance + cramps + hypoglycemia

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12
Q

Glucose-6-Phosphates - Function/Pathway/Disorder

A

Function - Converts Glucose-6P back to Glucose for export
Pathway - Glycogenolysis - Liver enzyme only
Disorder - Von-Gierke’s (Type 1 GSD) - AR - Severe fasting hypoglycemia + high blood lactate + hepatomegaly

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13
Q

Phosphoenolpyruvate Carboxykinase - Function/Pathway/Co-Factor

A

Function - Converts oxaloacetate to phosphoenolpyruvate - Primarily in the cytosol unless there is an NADH balance issue
Pathway - Gluconeogensis
Co-Factor - GTP

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14
Q

Fructose 1,6 Bisphosphatase - Function/Pathway/Regulation/Disorder

A

Function - Rate Limiting Step - Converts Fructose 1,6BP to Fructose GP - Opposite PFK-1 in Glycolysis
Pathway - Gluconeogenesis
Regulation - Increased by Citrate + Fructose 2,6BP (Which is turned on by Glucagon/Inhibited by Insulin)
Disorder - Extremely Low Glucose + High Lactate

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15
Q

Fructokinase - Function/Pathway/Disorder

A

Function - Converts Fructose to Fructose-1P (Traps it in the liver)
Pathway - Fructose Metabolism
Disorder - Essential Fructosuria - AR + A-symptomatic - High fructose in urine/blood

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16
Q

Aldolase-B - Function/Pathway/Disorder

A

Function - Converts Fructose-1P to Glyceraldehyde
Pathway - Fructose Metabolism
Disorder - Hereditary Fructose Intolerance - AR - Fructose 1P accumulates + decreases the availability of phosphate - decreases glycogenolysis + gluconeogensis (causes hypoglycemia) + vomiting + jaundice + hemorrhage + hepatomegaly - Tx is to remove fructose + sucrose

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17
Q

Aldose Reductase - Function/Pathway

A

Function - Converts glucose to sorbitol for trapping in some tissues
Pathway - Sorbitol Metabolism

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18
Q

Sorbitol DH - Function/Pathway/Disorder

A

Function - Converts Sorbitol to Fructose
Pathway - Sorbitol Metabolism
Disorder - Some tissue (retina + Schwann Cells + Kidneys) don’t have enough - Accumlation can cause osmotic damage (E.g. cataracts) - Associated with the diabetes based damages

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19
Q

Galactokinase - Function/Pathway/Disorder

A

Function - Converts Galactose to Galactose-1P (Uses ATP)
Pathway - Galactose Metabolism
Disorder - Galatokinase Deficiency - AR - Galactose can bypass pathway to become Galactitol via Aldose Reductase - Relatively mild

20
Q

Galactose-1-Phosphate Uridyltransferase - Function/Pathway/Co-Factor/Disorder

A

Function - Converts Galactose-1P to Glucose-1P
Pathway - Galactose Metabolism
Co-Factor - UDP
Disorder - Classic Galactosemia (GALT Deficiency) - AR - Causes galactosuraia + vomiting + diarrhea + jaundice - Galactitol Accumulation leads to symptoms mimicking severe diabetes (nerve/liver/retinal damage)

21
Q

PRPP Synthetase - Function/Pathway

A

Function - Serves as the Sugar/Phosphate and add bases to form Purines or modify bases to form Pyrimidines
Pathway - Nucleotide Synthesis

22
Q

PRPP Amidotransferase Function/Pathway/Regulation

A

Function - Rate limiting step in the conversion of PRPP to IMP
Pathway - Purine Synthesis
Regulation - Inhibited by IMP/AMP/GMP + Activated by PRPP

23
Q

IMP DH - Function/Pathway/Regulation/Co-Factor/Disorder

A

Function - Converts IMP to GMP
Pathway - Purine Synthesis
Regulation - Activated by High AMP - Inhibited by High GMP
Co-Factors - Glutamate + NAD+ + ATP
Disorder - Inhibited by the Drug Ribovarin - (Antiviral for HepC)

24
Q

Adenylsucinate Synthase - Function/Pathway/Regulation/Co-Factor

A

Function - Converts IMP to AMP
Pathway - Purine Synthesis
Regulation - Activated by GTP and Inhibited by ATP
Co-Factors - Aspartate + GTP

25
Q

Dihydrofolate Reductase DHFR - Function/Pathway/Disorder

A

Function - Restores THF which provides the 1-Carbons for Purine + TMP Production
Pathway - Multiple (Including Nucleotide Synthesis)
Disorder - Target of Methotrexate - DHFR Analog to prevent THF regeneration

26
Q

APRT - Function/Pathway/Co-Factors

A

Function - Salvage of Adenine and PRPP to AMP
Pathway - Purine Salvage
Co-Factors - Needs PRPP

27
Q

HGPRT - Function/Pathway/Co-Factors/Disorder

A

Function - Salvage of Hypozathine and Gaunine to IMP and GMP
Pathway - Purine Salvage
Co-Factors - PRPP
Disorder - Lesch-Nyhan Syndrome - HGPRT Deficiency - XR - Hyperuricemia + Gout + Pissed Off (Self-mutilation) + Retardation + DysTonia ——— Treatment - Allopurinol (GMP/IMP is the problem)

28
Q

AMP Deanimase (ADA) - Function/Pathway/Disorder

A

Function - Converts Adenosine to Inosine (for conversion to IMP) for degradation
Pathway - Purine Degradation
Disorder - ADA Deficiency - AR - Can’t break down dATP and ATP - Massive pools of ATP inhibit nucleotide synthesis - Decreases lymphocyte count and causes SCID (leads to infant death)

29
Q

Xanthine Oxidase - Function/Pathway/Disorder

A

Function Converts Hypoxanthine to Xanthine and Xanthine to Uric Acid (Second step is rate limiting)
Pathway - Purine Degradation
Disorder - Blocked by Allopurinol

30
Q

Ribonucleotide Reductase - Function/Pathway/Co-Factor/Disorder

A

Function - Converts to dexoxy state for DNA synthesis (activated in G1/S Phase)
Pathway - Nucleotide Synthesis
Co-Factor - NADPH
Disorder - Target of Hydroxyurea

31
Q

Carbamoyl Phosphate Synthase (CPS II) - Function/Pathway/Regulation

A

Function - Rate Limiting Step in Pyrimidine Synthesis - Forms the start of the base (Orotic Acid) to link with PRPP - Uses Glutamine + Aspartate
Pathway - Pyrimidine Synthesis
Regulation - Activated by ATP and PRPP and Inhibited by UTP/CTP

32
Q

UMP Synthase - Function/Pathway/Disorder

A

Function - Combines Orotic Acid + PRPP - Forms UMP
Pathway - Pyrimidine Synthesis
Disorder - Orotic Aciduria - Excess Orotate + Anemia - Treat with UMP/CMP

33
Q

Thmidylate Synthase - Function/Pathway/Co-Factor/Disorder

A

Function - Builds dTMP from dUMP (dUMP forme from either dCMP or UTP)
Pathway - Pyrimidine Synthesis
Co-Factor - Folate (THF)
Disorder - 5-Fluorouracil (5-FU) Inhibits it prevent TMP formation - Prevents DNA Synthesis - Anti-cancer drug

34
Q

Glucose-6P DH - Function/Pathway/Regulation/Co-Factor/Disorder/Disorder Causes

A

Function - Rate Limiting Step - Converts Glucose-6P to Ribulose-5P + CO2 + 2NADPH
Pathway - HMP Shunt
Regulation - Insulin Activates (Dephos.) + NADPH Inhibits
Co-Factor - NADP+
Disorder - XR - Favaism - No HMP - No Glutathione Restoration - Oxidative Damage + Hemolytic Anemia - Classes (I-IV with I being most severe) African = III vs. Mediterranean (II)
Disorder Causes - Sulfa Drugs + Antimalarials (Primaquine) + Fava Beans

35
Q

Phosphopentose Isomerase Transketolases - Function/Pathway/Co-Factor/Deficiency

A

Function - Converts Ribulose-5P to Glyceraldehyde-3P + Fructose-6P + Ribose-5P
Pathway - HMP Shunt
Co-Factor - B1 (Thiamine)
Deficiency - Wernicke-Korsakoff - Can’t use TPP - Ataxia + Confusion + Optha

36
Q

Glutathione Reductase - Function/Pathway/Regulation/Co-Factor

A

Function - Restores Glutathione after it reduces a peroxide to prevent free radical damage
Pathway - Oxidative Burst/HMP Pathway
Regulation - NADPH
Co-Factor - Uses NADPH to NADP+ and Requires Selenium

37
Q

Citrate Lyase - Function/Pathway/Co-Factor

A

Function - Converts Citrate from Mito. into Acetyl CoA and Oxaloacetate in the Cytoplasm (Citrateleaves due to high energy/inhibited Isocitrate DH)
Pathway - Fatty Acid Synthesis
Co-Factor- Requires ATP

38
Q

Acetyl CoA Carboxylase - Function/Pathway/Regulation/Co-Factors

A

Function - Rate Limiting Step of FA Synthesis - Converts Acetyl CoA to Malonyl CoA
Pathway - Fatty Acid Synthesis
Regulation - Insulin + Citrate Activate vs. Glucagon + Palmitoyl CoA Inhibit
Co-Factors - ABC Carboxylase - ATP + Biotin + CO2

39
Q

Fatty Acid Synthase - Function/Pathway/Co-Factors

A

Function - Elongates Fatty Acids (After Malonly CoA) 2 Carbons at a time
Pathway - Fatty Acid Synthesis
Co-Factors - NADPH + Pantothenic Acid (B5)

40
Q

Fatty-Acyl-CoA Synthase - Function/Pathway/Co-Factor

A

Function - Removes Acetyl CoA’s from Fatty Acids
Pathway - Fatty Acid Degradation
Co-Factor - Uses ATP

41
Q

Carnitine Acyltransferase (CPT1) - Function/Pathway/Regulation/Disorder

A

Function - Rate limiting step of the Carnitine Shuttle to bring Acyl CoA into the Mito. for B-Oxidation
Pathway - Fatty Acid Degradation
Regulation - Inhibited by Malonyl CoA (Activates synthesis + inhibits degradation)
Disorder - Deficiency - Relatively rare + commonly associated with hypoketotic + hypoglycemia + high levels of blood carnitine + hepatomegaly

42
Q

Carnitine Acyltransferase (CPT2) - Function/Pathway/Regulation/Disorder

A

Function - Same as CPT1 - Caratine Shuttle - Inner mitochondrial membrane vs. outer + not rate limiting
Pathway - Fatty Acid Degradation
Regulation - None
Disorder - 3 Forms - Adult (Skeletal Muscle/Fatigue/Pain) //////// Severe Infantile Multi-system Form - (Onset in the first 6-24 months of life - Hypoketotic Hypoglycemia + Hepatomegaly + Cardiomyopathy) ////// Neonatal Lethal - Symptoms within hour of birth + respiratory failure + seizures + cardiomegaly

43
Q

Acyl-CoA DH (AD) - Function/Pathway/Disorder

A

Function - Removes the Acetyl CoA for use in the Mito.
Pathway - Fatty Acid Degradation
Disorder - MCAD vs. LCAD vs. SCAD - AR - Hypoketotic + hypoglycemia + hyperammoneia due to liver damage + limited gluconeogenesis

44
Q

Carbomoyl Phosphate Synthase 1 - Function/Pathway/Co-Factors/Regulated

A

Function - Begins the Urea Cycle (in Mito.) as the Rate Limiting Step - Creates the first major substrate taking NH3 from Glutamate
Pathway - Urea Cycle
Co-Factors - Uses 2 ATP + CO2 + N-Acetylglutamate (From Arginine)
Regulated - N-Acetylglutamate is the regulatory co-factor - Levels correspond with Arginine levels (High Protein = High Urea Need = High Arginine = High N-Acetylglutamate

45
Q

Arininosuccinate Synthase - Function/Pathway/Co-Factor

A

Function - Adds the Aspartate in the Urea Cycle (Cytoplasm) to add the second N for release
Pathway - Urea Cycle
Co-Factor - 1 ATP

46
Q

Arginase - Function/Pathway

A

Function - Final Step of the Urea Cycle - Releases Urea + Restores Ornithine for use in further cycles
Pathway - Urea Cycle

47
Q

Homocystine Methyltransferase - Function/Pathway/Co-Factor

A

Function - Converts Homocystine (After using SAM from Methionine) bak to Methionine
Pathway - Amino Acid Carbon Chain Elimination
Co-Factor - B12

48
Q

Cystathionine Synthase - Function/Pathway/Co-Factor

A

Function - Converts Homocystine to Cystine or Serine (2 Step Process)
Pathway - Elimination of Amino Acid Carbon Chains
Co-Factor - Pyridoxine (B6)

Z - M1 Biochem (4 decks)

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