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26 Cards in this Set
- Front
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GAGs
Fxn? Amino sugar? 2 Acidic sugar? 2 Bond? Exception? |
Bind large amt of water -> gel-like matrix
D-glucosaine, D-galactosamine D-glucouronic acid, L-iduronic acid Covalent bond; hyaluronic acid |
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Glycolysis Steps
Step 1? 4 Step 2? 2 Step 3? 5 Step 4? 2 Step 5? 2 Step 6? 5 Step 7? 3 Step 8? 2 Step 9? 3 Step 10? 4 |
Glucose -> G-6-P
Hexokinase or glucokinase (liver) Req ATP Traps glucose in cell G-6-P -> F-6-P Phosphoglucose isomerase F6P -> F1,6P PFK RLS Req ATP +AMP, +F-2,6,P by PFK-2 (Inactivated by PKA <- cAMP <- glucagon) Adolase F1,6P -> DHAP + G3P Triose phosphate isomerase DHAP <-> G3P G3P -> 1,3-BPG G3P dehydrogenase Generate NADH Inh by Hg -> -2 ATP production Disrupted by Arsenic -> 0 ATP production 1,3-BPG -> 3-PG Phosphoglycerate kinase Generate ATP 3PG -> 2PG Phosphoglycero-mutase 2PG -> PEP Enolase Inh by Flouride -> 0 ATP production PEP -> pyr Pyr kinase Generate ATP Inactivated by phosphorylation (via PKA <- cAMP <- glucagon) Activated by Protein phosphatase (via insulin) |
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Pyruvate metabolism
4 endproducts? via? PDH: catalyzes? 5 factors? Activated by? 3 Inh by? Sx? 3 PDH def: Patho? Sx? Tx? |
Alanine (via ALT)
OAA (via pyruvte carboxylase + CO2 + ATP) Acetyl-CoA, NADH, CO2 (via pyruvate dehydrogenase) Lactate + NAD (via lactate dehydrogenase) Pyruvate + NAD + CoA -> acetyl-CoA + CO2 + NADH Pyrophosphate, FAD, NAD, CoA, Lipoic acid NAD > NADH, ADP, Ca Arsenic: vomiting, rice water stools, garlic breath Pyruvate & alanine backup -> lactic acidosis Neuro Incr ketogenic nutrients: high fat, or lysine or leucine |
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PKD = pyruvate kinase def
Patho? 3 Sx? |
Dec ATP -> ion pumps not work on RBC -> cell dehydration & membrane fragility
Hemolytic anemia Myopathy Neurological damage |
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NADH shuttles
2 forms? Used for? Pathways? 5, 7 |
Glycerol-3-P & malate-aspartate
Carry NADH into mitochondria NADH pass e- to DHAP-> DHAP becomes Gly-3-P -> cross membrane -> pass to FAD/Gly-3-P become DHAP -> cross membrane NADH pass e- to OAA -> OAA becomes malate -> malate cross membrane -> pass e- to NAD+/malate becomes OAA -> OAA pass to asp -> asp cross membrane -> asp pass to OAA |
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Anaerobic glycolysis
Used primarily in which tissues? 6 Low O2 state? 4 ATP demand > supply? 1 Contribution to muscle metab? 3 |
RBC, kidney medulla, white muscle fibers, lens, cornea, retina of eye
Initial exercise in muscle Developing tissue Large tumors Tissues in hypoxic state Sk muscle during intense exercise Fast twitch muscle Initial pd of exercise Intense exercise |
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Lactate
Fates? 3 Converted to? Lactate acidodic states? 6 Need for ATP reflected by? |
Heart & kidney convert to pyr to skip glycolysis
Liver convert to glucose by gluconeogenesis -> back to circulation Shuttle to brain -> goes to neurons from astrocytes Tissue infarcts Severe anemia Shock Congenital defects in pyr dehydrogenase, TCA, oxphos, gluconeogenesis Large tumors (seen on PET scans) Bacteria [AMP] > sensitive [ADP] |
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Insulin
Made by? Mechanism? Anabolic fx? Measured with? Moves glucose into? Mnemonic? GLUT receptors 1-5 [glucose]: blood? CSF? Hypoglycemia? Major regulators? 1 Minor regulators? 4 M/A? |
Beta cells of panc
Glucose -> incr ATP -> close K channel -> depolarization -> opens Ca channel -> exocytosis of insulin Incr glucose transport, glycogen synth & storage, TG synth & storage, Na retention, protein synth, cell uptake of K C peptide for endogenous L-BRICK: Liver, brain, RBC, intestine, cornea, kidney 1: RBC, brain 2: beta islet cells, liver, kidney, SI 3: neurons 4: adipose, sk muscle 5: fructose -> sperm, testes 5 mM; 7-11 mM; 1-3 mM +: glucose +: amino acids, neural input, gut hormones -: epi Bind extracellular domain of α chain of R -> tyr kinase -> protein phosphatase -> phosphorylase kinase (inactive) + phosphorylase a->b (inactive) + glycogen synthase (active) + acetyl coA carboxylase (active) + HMG CoA reductase (active) |
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Glucagon
Synthesized by? Inactivated by? Where? Major regulators? 3 Minor regulators? 4 M/A? Relation to epi, cortisol? |
α cells of islet of Langerhans
Liver protease -: glucose, insulin +: amino acids +: cortisol, neural (stress), epi, gut hormones Gs -> incr cAMP -> activate PKA (by releasing reg subunits) -> phosphorylase kinase-P (active) -> phosphorylase b->a (active) + glycogen synthase-P (inactive) + acetyl coA carboxylase-P (inactive) + HMG CoA reductase-P (inactive) Epi: bind β-adrenergic R -> incr cAMP; specific R in liver Cortisol bind intracellular R -> activate genes |
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PEP carboxykinase
Used for? Regulatory elements? 3 |
Gluconeogenesis
Glucagon response elements: CRE I, II IRE (insulin) inh transcription |
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Carb digestion
Sucrose-isomaltase: Enzymes? bonds hydrolyzed? Glucoamylase? Lactase? Trehalase? |
Sucrase -> b/t glucose & fructose
Maltase -> α-1,4 bond Isomalatase -> α-1,6 bond α-glucosidase -> α-1,4 on non-reducing end maltase -> α-1,4 bond Lactase -> β-1,4 bond Glycohydroase -> sugar residue w/ lipids/proteins Trehalase -> α-1,1 bond |
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Lactose intolerance
Patho? 3 Dx test? 3 |
Lactose + lactic acid from bacteria -> draw water into gut -> diarrhea
Lactose tolerance test Hydrogen breath test Stool acidity test |
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Monosaccharide transport into cell:
By? B/c? Glucose? Fructose? Galactose? |
Facilitated diffusion or active transport b/c too polar
Glucose transporter Fructose facilitative transporter Galactose transport on same transporter as glucose |
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Pentose phosphate pathway
Active in? 5 Oxidative steps? 2 Regulated by? Fate of R5P? 2 Transkeltoase steps? 2 Transadolase steps? 1 Irreversible oxidative step? Fate? 3 Reversibel nonoxidative step? Fate? 2 |
RBC, liver, mammary gland, testis, adrenal
G6P -> 6PGδL + NADPH via G6P dehydrogenase 6PGδL -> R5P + NADPH via 6PGδL dehydrogenase Entry of G6P into pathway by [NADPH] R5P -> ribose-5P via isomerase R5P -> X5P via epimerase X5P + ribose-5P -> S7P + G3P X5P + E4P -> F6P + G3P S7P + G3P -> F6P + E4P G6P -> CO2 + NADPH + R5P R5P for nuc biosynth NADPH for reductive biosynthesis, oxidative protection R5P -> F6P or G3P Glycolysis intermediates |
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NADPH fxns: 7
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Reduction of oxidized glutathione
Cyt P450 monoxygenase FA synth FA chain elongation Chol biosynthesis NT synth Nuc synth |
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Fructose metabolism
Synthesized in which pathways? 2 Converted to? By? Cleaved to? By? Major source for? GLUT transporter? Essential fructosuria def of? Genetics? Sx? Intolerance def of? genetics? Sx? 6 Test? |
Pentose phosphate pathway, polyol pathway
F1P by fructokinase DHAP + G3P by aldolase B Sperm/testis, GLUT 5 Fructokinase; auto R; benign Aldolase B; auto R Malabsorption, vomitting, diarrhea, gassy, hypoglycemia, jaundice H2 breath test |
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Polyol pathway
Step 1? Step 2? Buildup sorbitol in? 3 |
D-glucose + NADPH -> NADP+ + sorbitol via aldose reductase
Polyol + NAD+ -> D-fructose + NADH via sorbitol dehydrogenase Lens, nerve, glomerulus |
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UDP-glucose
Formed by? 3 Precursor for? 4 |
G1P + UTP -> UDP-glucose + PPi via G1P uridyltransferase
Irreversible if PPi cleaved Glycogen, lactose, UDP-glucuronate, glucuronides |
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Metabolic routes of UDP-glucuronate? 6
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UDP-gluocse -> UDP-glucuronate
UDP-glucuronate ->: + bilirubin -> bili diglucuronide + steroids/Rx/xenobiotics -> glucuronides UDP-X5P Iduronate Proteoglycans/glycoproteins |
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Lactose synthesis:
Only in? When? Enzyme complex? |
Mammary glands during lactation
Lactose synthetase = galactosyltransferase + α-lactalbumin α-lactalbumin = protein that changes galactosyltransferase from adding sugars to proteins to making lactose |
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Galactose metabolism:
Step 1? Via? Dz? Step 2? Via? Dz? Step 3? Via? |
Gal -> Gal-1-P via galactokinase
Def in nonclassic galactosemia Gal-1-P -> G1P via galactose-1-P uridyltransferase Def in classic galactosemia UDP-gal -> UDP-glu via epimerase |
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Glycogen synth/degradation:
Bond along chain? Along branch? Synthesis from glucose: Step 1? Step 2? Step 3? Step 4? Degradation to glucose Step 1? Step 2? Step 3? Step 4? Muscle regulated by? 2 |
α-1,4; α-1,6
Glu -> G6P via hexokinase/glucokinase G6P->G1P via phosphoglucomutase G1P -> UDP-glu via UDP-glu pyrrophosphorylase UDP-glu -> glycogen via glycogen synthetase/ 4;6 transferase/ branching enzyme Glycogen -> small amt glucose via debrancher enzyme Glycogen -> G1P via glycogen phosphorylase G1P -> G6P via phosphoglucomutase G6P -> glucose via glucose-6-phosphatase (liver only) AMP activate directly; Ca thru phosphorylase kinase |
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GLYCOGEN STORAGE DZ
Type, defective enzyme, Organ, Sx 1-6 |
1: Von Gierke's
G-6-phosphatase Liver, kidney Hepatomegaly, severe hypoglycemia, ketosis 2: Pompe's α-1,4-glucosidase (lysosomal) All organs Cardioresp failure; death <2 3: Cori's Amylo-1,6-glucosidase Muscle, liver Mild hypoglycemia, mild hepatomegaly 4: Anderson's Branching enzyme Liver, spleen HSM, liver failure, death <2 5: McArdle's Muscle phosphorylase Muscle Cramps with exercise 6: Her's Liver phosphorylase Liver Mild hypoglycemia, mild hepatomegaly |
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Gluconeogenesis
Energy-dependent steps? 3 Enzymes? Starting compounds? From? 2 Substances req? Acq from? 3 How pyr -> PEP? Where glycerol enter? Enzyme? |
pyr -> PEP via Pyr carboxylase; PEP carboxykinase; req biotin, ATP, GTP
F-1,6-P -> F6P; via PFK-1 = F-1,6-bisphosphatase G6P -> glucose via G-6-phosphatase Lactate from Cori cycle Alanine from muscle breakdown NADH: from lactate -> pyr; mal -> OAA ATP: beta-oxidation Pyr: from lactate Pyr -> OAA via pyr carboxylase OAA -> PEP via PEP carboxykinase Glycerol -> G3P via glycerol kinase |
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Hyperglycemia tests:
Sx? 2 Crisis? 3 OGTT test: c/i? how? +? FBG test: how? +? H1AC levels indicate? 5 |
Polydipsia, polyuria
>600 mg/Dl HNKC PKA develop in T1DM before C/i: pregnant (but used to Dx gestational DM) Drink 75g cola -> blood glucose ever .5 hr x 2 hr >200 = + (need confirm) 8 hr fasting > 126 = + (need confirm) Norm: 4-6% <7: excellent sugar ctrl 7.1-8: good ctrl 8-9: fair ctrl >9: poor ctrl |
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Hypoglycemia
LLN? Sx? Infants? Response hormones? 2 |
<80 mg/dL
HA, dizziness, sweating, tremors, anx, confused speech, etc Premature have low glycogen storage -> more susceptible Cortisol, epi |
