Explain Low Energy State (LES) The body is in the business of staying alive, when anything interferes with

the system, the body begins to dies. The parts of the body that dies first are the mitotically active (or rapidly dividing) cells. Symptoms LES? Brain - Confusion Muscle - Weakness Lung (Cilia) Kidney (PCT) Skin - Dry and Cracked Hair - Brittle Cuticles GI Tract - N/V Bone Marrow - Suppressed Endometrium Atrophic Sperm count - Low Bladder - Hemorrhagic Cystitis Vascular - Vasculitis

MC:SSx/Infx/CODfor LES Sign Dyspnea/Tachypnea Symptom Weakness/SOB Infx Pulmonary/UTI COD - Heart Failure Only "Imino" acid? Proline Common with all Amino Acids? They all have: Amino & Acid group Define: Dissociate, Soluble Bioavailable Dissociation - Giving up H+ Soluble - Charged Bioavailable Uncharged Best AA buffer in bodies Histidine

Define Anode and Cathode Anode - Pole anions go to Cathode - Pole cations go What is the pH in the GI Stomach 1-2 Duodenum 3-5 Early Jejunum 5-7 Late Jejunum 7-9 Ileum >9 Define isoelectric point. Isoelectric Point means there is "No Net Charge" What does Ketogenic mean? Made from and broken into AcCoA (Avoid in DM) What does Glucogenic mean? Made from and broken into Anything but AcCoA.

For each of the following, give the Amino/Imino acid: Smallest - Glycine Kinks - Proline Basic - Lys, Arginine Acidic - Glu, Asp N-Bonds - Asn(99%), Gln O-Bonds - Ser, THr, Tyr Sulfur Cys, Met Aromatic - Phe, THr, Try Branched Chains LIV Ketogenic - Lys, Leu Both PITT Essential PVT TIM HALL Which amino acid is used to make cystein? Methionine Four hormones with disulfide bonds. Prolactin Insulin Inhibin

Growth Hormone Newborn Screening disease PKU CAH Biotinidase Grown Hormone Hypothyroidism Galactosemia Sickle Cell Enzyme deficiency in PKU Phenylalanine hydroxylase S/Sx of PKU Musky odor MR Fair Skin Blue Eyes Blonde Hair AA deficient in PKU

Tyrosine What Substances build up? Phenyl-Pyruvate Phenyl-Acetate Treatment for PKU Avoid Aspartame What Product has Aspartame? Nutriasweet Defect in MSUD Nephron Transport Protein S/Sx of MSUD Sweet Urine AA s in MSUD Leucine Isoleucine Valine

Defect in Cystein Urea Cystine Ornathine Lysine Arginine What crystal are seen in Cystein Urea Coffin lid/Envelope shaped Amino Acids humans have? L-Amino Acids What determines AA Primary Structure? Amino Acid Sequence What is the primary factor to determine Structure of Protein? Hydrophobic-Hydrophilic interaction What are 3 characteristics of peptide bonds? Planar - Flat Limited Rotation

Trans Configuration What is characteristic of amino acid quaternary structures? Allesterism (Cooperatively) For Enzymes, Define KM, Vmax. Km - Potency Vmax - Efficacy Km = Vmax Km = 1/Affinity How does noncompetitive INH work? Binds to active site. Km increases. Vmax stay the same How does noncompetitive INH work? Binds to Regulatory Site and Inactivates the protein Km stays the same. Vmax decreases.

Metabolized in Zero and first order substances Constant Amount Constant percentage Three 0 order substances Phenytoin EtoH ASA 0 order number for EtoH 100mg% or 100 [mg/dl]/hr) Define GABA connection? NH4 + aKG
Glu
GABA
Slows things down From what are porphorin rings made? Succinyl -CoA Rate Limiting enzyme in Heme Syntehsis? Delta - Amiolevulonic Acid Synthase(D-ala)

What are the structures of Hb A, A2, and F? A - a2b2 A2 - a2d2 F - a2g2 In what percentage does each exist in the body? A - 98% A2 - < 2% in F child < 6 mos. Enzymes INH by Lead in Heme Systhesis d-ALA/ Ferrochetalase What Enzyme adds Iron to porhorin rings? Ferrochetalase What buffers in Fe2+? HISTIDINE # of heme sites in 1g heme Four

Grams of iron in 1U blood 3.4 Acute Intermittent Porphoria Recurrent, Severe, Acute abdominal pain & Neuropathies Clues for Porphoria Cutanea tarda Blisters with light Onset > 5 yr Clues for Erythrocytic protoporphoria Blisters with light Onset < 5 yrs Rx for AIP Fluids, Sugar, Hematin MCC Aggravation of AIP Stress and Menses

Define Hemosiderosis and Hemochromatosis Hemosiderosis - Iron load Overwhelm BM Hemochromatosis - Iron Deposition in Organs What causes Primary Hemochromatosis? Duodenum is absorbing too much iron AR HLA A3 What causes secondary Hemochromatosis? Too many blood Transfusion Timeline for erythropoiesis Starts 4 mos. - Yolk Sac 6 mos - liver/Spleen/Flat B 8 mos - Long Bones 1yr - (-) Liver/Spleen/Flat B Wipe BM - Spleen opens up What is the defect in all the thalassemias? Gene Deletion

# genes for Alpha/Beta Alpha 4 Beta 2

Define thalassemia minor/major. Minor 1 gene left Major No genes Alpha Minor 1 gene Asymptomatic Alpha Minor 2 genes Active - Symptomatic Not Active - Asymptomatic Hb Bart Beta Tetramer Alpha Minor 3 genes Symptomatic Alpha Major 4 genes Hydrops Fealis

Beta Minor (+/-) SxS OK until 4-6 months Beta Thalassenia Major Always Symptomatic Transfused dependant Only Hg A2 and F Cooleys s Anemia What is Cooley s Anemia? Big Skull, Hepatoplenomegaly Big Sternum Big Pelvis What will the COD for Beta-Major? 1st 10 yrs Infections Teens Hemochromotosis Acids used in medicine? Mb, Phenobarbital, ASA,TCAs

Base used in Medicine Base - NaHCO3 Bases Used in Medicine Amphetamines Acid used for treatment in Hospital NH4Cl (9) causes of Right Shift of Oxy-Hb curve? Hypoxia Increased Lactic Acid Increased 2,3 DPG Increased [H] Increased Temp Increased Altitude Increased Exercise Decreased pH Increased PCO2

Released from muscle cells during Rhabdomyolysis? Myoglobin Fetal circulation: 100% - From Placenta 80% - Umbilical Vein + blood from feet via Ductus Venosum 60% - Inferior Vena Cava 50-53% - Right Atria 80% goes Foremen Ovale
Left Atria 20% goes Right Ventricle
Pulmonary Artery 20% - Lower Extremities
Umbilical Arteries
Placenta Carbon Monoxide Competitive Inhibitor Binds 200x stronger than O2 SaO2 is High PO2 is Low

Rx for CO2 poisoning? Hyperbaric O2 Cyanide Non competitive Inhibitor PO2 is normal SaO2 is Low MCC Cause of CN poisoning? Sodium Nitroprusside used for Hypertensive Crisis Rx for CN poisoning? Amylnitrate - Fe2+
Fe3+ Thiosulfate
Thiocyanate Methylele Blue Fe3
Fe2 Rx methemoglobinemia Methylene Blue What causes primary methemoglobinemia?

Congenital What causes Secondary Methemoglobinemia? Drugs MC sulfa What is the PO2 and SaO2 for methemoglobinemea? PO2 is Normal SaO2 is Low Effect of acid hydrolases on proteins? Denatures Protein Change ASN
ASP Change Glutamate to Aspartic acid and glutamic acid How does GEL electrophoresis work? Separates by size Separates by charge How does the Ninhydrin reaction work? Turns AA purple Turns Proline Yellow

How does the Edmon's degeneration work? Uses Phenyl/Iso Thiocyanate 1 AA at a time from Amino Only good for 100 AA. Trypsin Cuts to right of Lysine, Arginine Chymotrypsin Cuts Phenylalanine, Threonine, Tyrptophan Elastase Cuts (Hlycine, Alanine, Serine) CyanatobromideCuts (Methinonine) Aminopeptidase Cuts (Amino Terminal) Carboxypeptidase Cuts

(Carboxy Terminal) Only that cuts to the left. Mercaptoethanol (Dissolves disulfide bonds) 4 most abundant collagen I - S - Skin and Bone II -C - Connective Tissue III -A - Arteries IV -B - Basement Membrane What protein in defective in Marfans? Fibrin Protein Clues for Marfan Wingspan > height Arachondactyly Lens disloc from bottom Clues for Ehrlers-Danlos? Hyperstretchable Skin

How does Homocystein work? INH lysine hydroxylase
INH collagen synthesis Clues for Homocysteinurea Dislocate lens from top What is the defect in scurvy? Vit C is needed to hydroxylate Lsy and Pro in Collagen Synthesis Clues for Scurcy Gingival Bleeding Perfolicular Hemorrhage Clues for Syphilis Obliterative Endarteritis Tree barking Shooting, Lancinating Pain Clues for Congenital Syphilis Anterior bowing of legs

Snuffles Huthinson's teeth Defect in Minky's Kinky Hair disease? Cu2+ deficiency Clues for Minky's Kinky Hair looks like copper Hair cuts face Name the disease Dilocation from Top Dislocation from botton Perifolicular hemorrhage Blue Sclera Copper looking hair Tree barking of aorta Hyper-stretchabls skin Pulseless Aortitis Differentiate OI from abuse OI
Shattering fracture

In what population do you see Takayasu disease? Asian Women What is Takayasu? Granulomatous Aortitis What is the only protein modified in the ER? Collagen What are the Amino acids found in collagen? Gly, Pro, Lys What vit is needed for collagen synthesis? Vitamin C What other mineral is required for collagen? Copper What two A.As are Hydroxylated in Colagen?

Proline and Lysine How do all proteins begin? Pre-Pro-Protein In what form is collagen secreted? What happens after secretion? Collagen is secreted from the GOLGI as Tropocollagen and a cytoplasmic Peptidase Cleaves off OH To what structure does each of the following guide a protein? Where do each of the following guide a protein? Pre- ER Pro - Golgi Man-6-Phos - Lysosomes Short Amino Terminus Sequence mitochondria Diff bt. elastin & collagen?

Elastin has no OH-Lysine. What gives Elastin elasticity? Desmosine
(4) Lysines

Enzyme breaks down Elastin? Elastase 2 bacteria with elastase? Staph , Psuedomonas How does the body INH elastase? With antitrypsin Define Emphysema? Cystic Dilation of the alveoli 4 types of emphysema Pan-acinar Antitryp def Centri-acinar - Smoking Disto-acinar - Age

Boullous

Staph/Pseudo

Another name for boullous Pneumatocele What providesTensile strength? Keratin Kinds of bonds in Keratin Disulfide bonds 5 Actions of Enzymes Bring [S] together Stabilizes HEI Lower free EOA Not consumed Reaction goes faster ETC complex needing Fe? Cpmplex III and IV ETC complex needingCu? Complex III

FADH2/NADH feed ETC at FADH2
Complex II NADH
Complex I InhibitingETC means? Stop electron transport & Stop Heat generation. Uncoupling ETC means? Stop electron transport, but Heat generation continues ETC Inhibitors I - Amytal, Rotonone II - Malonate III - Actinomycin D IV - CN , CO, Chloramphenical V - Oligomycin ETC Uncouplers DNP - Dinitrophenol

ASA - Aspirin FFA Who has uncoupled ETC Babies & Polar Bears Naming enzymes 1st name - Substrate Last name Action Type of Sugars & AA in humans? L-Sugars D-Amino Acids Why does the body phophorylate things? To keep them in the cells What are the regulatory enzymes in Glycolosis? Hexokinase, PFK1, Pyruvate Kinase Where will you find

Glucokinase is found where? Liver & Pancreas after meal Hexokinase is found where? Everywhere

List 6 stress hormones Epinepherine - Immediately Glucagon - 20 minutes Cortisol - 2-4 hours GH - 24 hours Insulin - hour ADH with
Osm 7 not requiring Insulin to Take up Glucose: B - Brain R- RBC I - Intestinal Wall C - Cornea K - Kidney L - Liver

E - Exercising Sk. Muscle 3 enzymes needing B1 Pyruvate Dehydrogenase Alpha KG Dehydrogenase Brached chain AA DH

List the B vits and names B1 - Thiamin B2 Riboflavin B3 - Niacin B4 Lipoic Acid B5 Pantothenic Acid B6 - Pryladoxmine B9 - Folate B12 - Cyanocobalomine List Cofactors for B vits B1 - TPP B4 Lipoic Acid B5 CoA

B2 FAD B3 NAD List Deficiency for vits A Vight Blindness B1 Beri Beri B2 Angular Chielosis B3 Pallagra B4 None B5 None B6 Neuropathy B9 Megaloblastic Anemia B12 Megaloblastic Anemia with neuropathy C Scurvy D Osteomalasia/Rickets E K Bleeding disorder Clue for Pellagra Dermatitis Diarrhea Dementia

Death Disease like pellagra Hartnup What are sources of vits B2 - milk What breaks down B2 Sunlight What cannot be transported in Hartnup? Tryptophan 5 fates of Pyruvate Lactic Acid Alanine OAA AcCoA EtoH Enzyes for 5 fates of pyruvate

Lactate Dehydrogenase Alanine Transferase- ALT Pyruvate Carboxylase Pyruvate Dehydrogenase Ethanol Dehydrogenase What is LDH1? Marker for cardiac damage

What is an LDH Flip? 5 LDH s exist. LDH1 is lowest. With MI LDH 1 is highest Viral vs- EtoH hepatitis Viral 1:1 AST:ALT. Alcoholic > 2:1 AST:ALT. Explain AST: ALT ratio AST in cyto & mitochondria ALT only in the cytoplasm. Viruses
membrane
1:1

EtoH
also Mitochondria
> 2:1 Most specific cardiac marker Troponin I MI Markers T - 2hr P 2d L 7d L - 6hr P 12hr L 24 hr L 24hr P 48hr L 72 hr Drugs causing disulfrm rxn Disulfram and Mitronidazole INH & Act (PFK1) (+) F 1,6BP, F2,6BP (-) Citrate INH & Act Pyruvate carboxylase (+) Acetyl-CoA (-) Glucose INH & Act CPS1 (+) N-AcetylGlutamine

(-) NO INH & Act Glycogen Alpha 1,4 Glucosyltransferase INH & Act G6PD (+) G6P (-) Ribose-5-P INH & Act Acetyl CoA Carboxylase INH & Act Fatty Acyl Phosphorylase INH & Act HMG-CoA Synthase INH & Act HMG-CoA Reductase INH & Act PRPP Gln Aminotransferase Mercury Inhibits? G3P Kinase Floride Inhibits?

Enolase Signs of Floride poisoning? Pearly white teeth & bones 6 TCA locations for AA s: Pyruvate Acetyl-CoA Alpha - Keto-Glutyrate Succinyl-CoA Fumerate TCA enzyme connected to ETC Succinyl-CoA Dehydrogenase. 2. It is connected at Complex II Intermediates in Glycolysis Glucose G6P F6P F16P BHAP/GA3P 1,3 Bis Phosphogluconate

3 PG 2 PG PEP Pyruvate Enzymes in glycolysis Glucokinase/Hexokinase Glucose-6-P Isomerase Fructose-6-P Kinase Aldolase A GA3P Kinase 1,3 Bisphosphoguconate DH 3PG Isomerase Enolase Pyruvate Kinase

4 regulatory enzymes in gluconeogenesis Pyruvate Carboxylase PEPCK Fructose 1,6 Bisphosphatase Glucose-6 Phosphatase

Intermediates in TCA Cindy Is Kinky So She Fornicates More Often. Acetyl-CoA Citrate Isocitrate Alpha-Keto-Gluterate Succinyl-CoA Succinate Fumerate Malate Oxaloacetate Intermediates in Urea Cycle

OJ (Ornathine) took the CAR to the City to get some Asp he argued and Fumed, argued and Fumed and was left alone. NH4 + CO2 + ATP Carbamoyl-P+ Ornithine Citruline +Asp Arginosuccinate -Fumerate L-Arginine -Fumerare Ornathine + Urea Intermediates in Pentose Pathway Glucose Glucose-6-P 6Phosphogluconate Ribose-5-P Intermediates in the Fructose catabolism Fructose Fructose-1-P BHAP/Glycerol Deficiency in Fructosurea Fructokinase Deficiency in Fructosemia

Aldolase B Deficiency in Galactosurea Galactokinase Deficiency in Galactosemea Galactose-1-Urudyl- Transferase Complications of Fructosurea/Galactosurea Polydipsia Polyurea UTI

MCC of UTIs? E. Coli Proteus Klebsiella Candida Carriers in body UDP Single Sugar

Dolichol Many Sugars Biotin CO2 THF - CH3 for Nucleotide SAM CH3 for everything CDP - AA Excess sugars Glu
Sorbitol Fructose
Fructicol Galactose
Galacticol