My Biochem Cards

Front 1

Hemolytic anemia causes

Back 1

Pyruvate kinase >> phosphoglucose isomerase

Front 2

Glycolysis steps regulated (3)

Back 2

1 = RDS (PFK), regulated by ATP/citrate/AMP/F26BP; 2 = Pyruvate Kinase, regulated by ATP/alanine/F16BP;3 = Pyruvate deH, regulated by ATP/NADH/acetyl-CoA

Front 3

Things required by pyruvate deH (5)

Back 3

B1/2/3/5 (thiamine, riboflavin, niacin, panthenate) + lipoic acid (which is inhibited by arsenic); activated by exercise (NAD, ADP, Ca2+)

Front 4

Arsenic

Back 4

Blocks lipoic acid mediation of Pyruvate deH rxn; rice water stools, garlic breath, vomiting

Front 5

Pyruvate deH deficiency

Back 5

Pyruvate/alanine buildup -> lactic acidosis; tx with ketogenic diet (LL Kool Jenic, leucine/lysine)

Front 6

Pyruvate options (4)

Back 6

1 = replenish NAD+ for glycolysis (lactate production);2 = accept NH3 group and become alanine (off to liver for urea production);3 = oxaloacetate (glucogengenesis or TCA intmd);4 = Acetyl-CoA (into TCA; produces CO2 and NADP)

Front 7

Cori cycle

Back 7

Taking lactate from muscles/RBCs, turning it into pyruvate/glucose in the liver (costs 18 ATP), then shuttling back in blood

Front 8

Alpha KG deH

Back 8

converts alphaKG into succinyl CoA; Very similar to pyruvate deH (requires same factors); also produces CO2 + NADP; inhibited by ATP/NADH/Succinyl CoA (again, very similar to Pyruvate-deH);

Front 9

Oligomycin

Back 9

Blocks flow of H+ thru ATPase (increases H+ gradient but decreases ATP production)

Front 10

Irreversible step(s) of gluconeogenesis that take place in the mitochondria

Back 10

Pyruvate->oxalate (pyruvate carboxylase); requires biotin/ATP; promoted by acetyl-CoA

Front 11

Irreversible step(s) of gluconeogenesis in the cytosol

Back 11

1: PEP carboxylase converts oxaloacetate to PEP (requires GTP);2: Fructose-1,6-BP converted to F6P by Fructose-1,6-bisphosphatase;

Front 12

Irreversible step(s) of gluconeogensis in the ER

Back 12

G6P dephosphorylated

Front 13

Where can gluconeogenesis occur?

Back 13

Kidney, gut epithelium, LIVER

Front 14

Things that contribute substrates to gluconeogenesis (4)

Back 14

1: lactate; 2: alanine (protein catabolism); 3: Glycerol from adipose tissue; 4: metabolic product (propionyl-CoA) of odd (literally odd-numbered)-chain fatty acids

Front 15

PPPathway (HMP Shunt)

Back 15

Alternative to glycolysis for G6P; either oxidative rxns or non-oxidative rxns; these are "free" rxns in the cytoplasms that do not consume/generate ATP; most important in sites of synthesis (mammary, liver, adrenal cortex) and RBCs

Front 16

Oxidative rxns in PPP

Back 16

Key enzyme = G6DP; irreversible; creates NADPH for FA/steroid synthesis and GSSH reduction;

Front 17

Non-oxidative rxns in PPP

Back 17

Reversible; create glycolytic intermediates (G3P/F6P) and R-5-P for NT synthesis; requires thiamine

Front 18

Fructose intolerance

Back 18

aldolase B deficiency; causes PO4 "sink" blocking neogenesis, glycogenolysis -> hypoglycemia, jaundice, cirrhosis; must avoid fructose (and sucrose)

Front 19

Essential fructosuria

Back 19

Can't even metabolize fructose due to fructokinase deficiency; benign condition

Front 20

Defect in galactose-1-P uridyltransferase

Back 20

Galactosemia; toxic buildup of galactose metabolites -> cataracts/MR/hepatomegaly; avoid galactose (and lactose) in diet

Front 21

Defect in galactokinase

Back 21

Mild galactosemia; galactitol may appear in urine if galactose ingested

Front 22

(exclusively) glucogenic amino acids

Back 22

Met Val Arg His

Front 23

Gluco+ketogenic amino acids

Back 23

Phenylalanine, Tyrosine, Tryptophan, Isoleucine

Front 24

How protein-nitrogen gets ready for elimination

Back 24

Aminoacids donate NH3 to create glutamate, which hands off NH3 to pyruvate (becomes alanine); alanine travels to liver, where it hands off NH3 to create glutamate, which gives NH3 to urea

Front 25

How NH4 gets turned into urea

Back 25

Urea cycle: NH4+CO2+2ATP --Carbamoyl phosphate synthase---> CP; CP starts cycle; aspartate enters cycle at some point, and arginine created at some point; end produce of cycle (ornithine) turned into citrulline

Front 26

Arginine -->

Back 26

Creatinine, urea, Nitric oxide

Front 27

Phenylalanine-->Tyrosine ->

Back 27

Thyroxine, DOPA (melanin, DA, NE, EPI)

Front 28

Glutamic Acid

Back 28

GABA (B6-required) and GSH

Front 29

Phenylacetate/lactate/pyruvate in urine

Back 29

Phenylketonuria (missing phenylalanine hydroxylase), which leads to toxic metabolite buildup + tyrosine becomes essential AA; MR/Growth stunt/musty (aromatic) odor

Front 30

Homogentisic acid oxidase

Back 30

Deficiency causes black urine upon standing and black CT (alkaptonuria); may cause joint pain, but benign otherwise

Front 31

Albinism causes

Back 31

Can't get tyrosine into cell or can't convert tyrosine into melanin; failure of NCC migration; skin cancer risk inc.

Front 32

Homocystinuria (1/2/3)

Back 32

1: can't convert HS into cysteine (cystathionine deficiency/B6); fix by upping cysteine/B12 while decreasing met in diet; 2: decreased affinity of the same enzyme; tx by upping B6; 3: can't convert HS into met (HS methyltransferase deficiency)

Front 33

General principles of homocystinuria

Back 33

Cysteine becomes essential AA (up diet); MR/osteoperosis/tall stature/atherosclerosis/lens falls into PC

Front 34

Cystinuria

Back 34

PCT transporter defect; cysteine (hexagonal) stones; tx by alkalinizing urine (acetazolamide) to reduce S-S bonds and inc. solubility

Front 35

Maple syrup Dz (alpha ketoacid deH, not to be confused with alpha ketoglutarate deH in the TCA)

Back 35

Certain aa's (branched) can't be degraded; in urine, they smell like maple syrup; MR/CNS/death; aa's = isoleucine/valine/leucine;I(soleucine) L(eucine) V(aline) --> ILV = I Love Vermont Maple Syrup!

Front 36

AMP broken into adenosine…how does adenosine get back to AMP?

Back 36

Adenosine -> inosine -> hypoxanthin -> IMP -> AMP

Front 37

GMP broken into guanosine…how does it get back to GMP?

Back 37

Guanosine -> guanine -> GMP

Front 38

Important enzymes (2) in the AMP cycle

Back 38

1: AID converts adenosine to inosine; AID deficiency causes SCID (bubble boy) syndrome; when AMP builds up, ATP/dATP made in excess such that the enzyme that removes the 2' OH group from nucleotides (ribonucleotide reductase) gets inhibited;2: HGPRT converts hypoxanthine to IMP; defect leads to buildup of xanthine/uric acid

Front 39

Lesch-Nyhan (purine salvage)

Back 39

Self-mutilation, gout, retardation, aggression; HGPRT mutation (X-linked);

Front 40

AID Deficiency (purine salvage)

Back 40

Can't convert adenosine to inosine, leading to shutting down of NT synthesis and severe (no B/T cells) immunodeficiency; X-linked

Front 41

First 4 things the body does (over the first 3 days of food deprivation)

Back 41

Use up current supplies (glycogenolysis, gluconeogensis); start breaking down fatty acids; muscle/liver shift to using fat as fuel source; hepatic gluconeogenesis using catabolic substrates (i.e. alanine) and fat products (odd chain breakdown + glycerol)

Front 42

Insulin-sensitive glucose transport

Back 42

GLUT4 in muscle/fat

Front 43

GLUT1

Back 43

Brain/RBC

Front 44

GLUT2

Back 44

Pancreas, kidney, liver

Front 45

Electrolyte FX of insulin (2)

Back 45

Shift K+ into cells and increase Na+ retention at kidneys

Front 46

Glycogen defects: I

Back 46

Glucose-6-phosphatase missing so liver can't release glucose; hepatomegaly/hypoglycemia; blood lactate increased (acidosis); Von Gierke's

Front 47

Glycogen defects: II

Back 47

Heart eruption (Pompeii); cardiomegaly caused by inability to breakdown residual glycogen in lysosomes (a1,4 glucosidase); leads to early death

Front 48

Glycogen defects: 3

Back 48

Cori; missing a-1,6 debranching enzyme, so glycogen is useless; mild version of type 1 (lower lactate levels)

Front 49

Glycogen defect: 5

Back 49

McArdle's in the muscle; missing glycogen phosphorylase, so can't break down glycogen; exercise intolerance that can lead to myoglobinuria

Front 50

Form of glucose that is added to glycogen

Back 50

Glucose-UDP

Front 51

Hepatosplenomegaly, the bends of femur, macrophages that look like crumpled tissue paper

Back 51

Buildup of glucocerebrosidase (Beta enzyme deficiency), mc problem; Gaucher's

Front 52

Tay-Sach's

Back 52

Tay SaX (hexosamidase defect -> GM2 buildup); cherry red spot on retina, MR, onion lysosomes; Ashkenazi Jews

Front 53

Neimann-Pick (look at the belly!)

Back 53

Sphingomyelin buildup; FTDementia; also seen with cherry red spots, but it is macular in location; also, GI MEGALY; with foam cells

Front 54

Krabbe's

Back 54

Confluent loss of WM (demyelination); buildup of ceramides (defect in galactocerebrosidase); peripheral neuropathy + globoid cells + optic atrophy

Front 55

Ashkenazi roundup:

Back 55

GI megaly + cherry spot = Pick's

Front 56

GI megaly + cherry spot =

Back 56

GI megaly + bone findings = Gaucher's

Front 57

GI megaly + bone findings =

Back 57

Cherry spot + developmental delay = Tay-Sach's

Front 58

Cherry spot + developmental delay =

Back 58

tay sachs

Front 59

Central and peripheral nervous findings; ataxia + dementia

Back 59

Arylsulfatase deficiency (cerebrosidase accumulation) seen in MLD

Front 60

Corneal clouding (anterior eye finding) + gargoylism + airway obstruction (breathe!) + dev't delay

Back 60

Mucopolysacch def (Hurler's); iDuron (alpha deficiency); heparan sulfate builds

Front 61

Mild gargoylism + aggressive = awesome! No clouding of corneas

Back 61

Hunter's; X-linked; iDuron (iduron sulfatase)

Front 62

X-linked storage Dos

Back 62

Hurler's, Fabry's

Front 63

Renal glomerular findings; purple splotches; LVH; corneal crap; peripheral neuropathy

Back 63

Fabry's (alpha galactosidase, ceramide trihexoside)

Front 64

Familial hyperlipidemias (I, IIa, IV)

Back 64

1: elevated chol/TG (chylomicrons up due to LPL/CII defect);2a: elevated chol due to decreased LDL receptors at liver;4: elevated TG due to increased VLDL production

Front 65

apoAI

Back 65

Activates LCAT

Front 66

Apo48

Back 66

Chylomicrons

Front 67

Essential fatty acids

Back 67

Linoleic acid and linolenic acid (need these for eicosanoids)

Front 68

Roles of citrate and carnitine in fatty acid metabolism

Back 68

Citrate shuttles Acetyl-CoA out of the mitochondria for FA synthesis; carnitine brings long-chain FAs into the mitochondria for beta oxidation

Front 69

HMG CoA --> mevalonate

Back 69

Catalyzed by HMG CoA Reductase (statins)

Front 70

Malonyl-CoA

Back 70

Intermediate in FA synthesis; inhibits shuttling of FAs back into mito for destruction

Front 71

Pb2+ FX on heme synthesis

Back 71

Blocks 2 steps in mitochondria: ALA dehydratase (export to cytoplasm) and ferrochelatase (adding Fe to protoporphyrin)

Front 72

Acute intermittent porphyria

Back 72

Can't convert urobilinogen to uroporphyrinogen in cytoplasm

Front 73

Porphyria cutanea tarda

Back 73

Can't get things back into mitochondria (decarboxylase enzyme affected);

Front 74

Tea colored urine; dALA + Coproporphyrin; dALA + Prophobilinogen

Back 74

Cutanea tarda, Pb poisoning, acute intermittent porphyria

Front 75

General problem with porphyrias

Back 75

Not enough heme --> microcytic anemia; intermediate accumulation leads to clinical symptoms (PPPPP);Abd pain!;Urine findings (pink);Polyneuropathy;Psyche changes;Precipitation via drugs

Front 76

Heme--> _____ --> ______ -->

Back 76

Heme, biliverdin, bilirubin, urobilinogen, urobilin (renal excretion)

Front 77

Aldolase B vs Aldolase Reductase

Back 77

Aldolase B is defective in Fructose intolerance (the worse of the 2 fructose problems); Aldose reductase is the lesser of the two galactosemias (galactokinase deficiency)

Front 78

Thiamine-dependent (B1)

Back 78

Non-oxidative rxns of PPP; production of acetyl CoA (pyruvate deH); production of succinyl-CoA (alphaKG deH); branched-chain AA degradation (maple syrup Dz, alpha ketoacid deH def)

Front 79

Biotin-dependent (decarboxylation)

Back 79

Pyruvate --> oxaloacetate (Pyruvate carboxylase for gluconeogenesis); malonyl-CoA --> fatty acid synthesis in cytoplasm;Production of MethylmalonylCoA from propionyl-CoA

Front 80

Carbamoyl Phosphate Synthase

Back 80

RDS of urea cycle

Front 81

Three uses of NADPH

Back 81

Steroid synthesis, GSH reduction, P450 metabolisms

Front 82

Steps in Glycolysis that cost ATP

Back 82

Hexokinase and PFK-1

Front 83

Steps in glycolysis that produce ATP

Back 83

Phosphoglycerate kinase and pyruvate kinase (the step that makes pyruvate)

Front 84

Steps of glycolysis which are regulated

Back 84

3rd (RDS) and the last two (pyruvate creation and acetyl-CoA creation)

Front 85

B6 depletion or carcinoid syndrome may cause which clinical symptoms?

Back 85

Pellagra (diarrhea, dermatitis, mental changes); Niacin deficiency

Front 86

B6 deficiency

Back 86

Irritability, anemia, convulsions

Front 87

B5 deficiency

Back 87

Alopecia, dermatitis, enteritis

Front 88

B2 deficiency

Back 88

Cheilosis, corneal vascularization, angular stomatitis (FACE)

Front 89

B1 def

Back 89

Wernickes or Beri Beri (Wet = dilated CHF, dry = neuropathy, symmetrical muscle atrophy)

Front 90

Anything involved CoA also involves…

Back 90

B5 (pantotheone)

Front 91

VitC helps collagen, but also (2)

Back 91

Helps absorb Fe2+ and helps convert DA to NE

Front 92

VitE

Back 92

Antioxidant in RBCs

Front 93

Less hormone-induced hair, hypogonadism

Back 93

Zinc deficiency

Front 94

B6 uses

Back 94

Making heme, glycogen phosphorylase; plays a role in homocysteinuria (helps overcome enzyme affinity decrease)

Front 95

HS + CH3 --> _____, mediated by what

Back 95

Methionine, mediated by THF-CH3 + B12

Front 96

B12 deficiency causes neuropathy when what accumulates

Back 96

Met down or methylmalonyl-CoA up (can't enter Kreb's cycle at succinyl CoA)

Front 97

Steps in glycolysis that produce ATP

Back 97

Phosphoglycerate kinase and pyruvate kinase (the step that makes pyruvate)

Front 98

Steps of glycolysis which are regulated

Back 98

3rd (RDS) and the last two (pyruvate creation and acetyl-CoA creation)

Front 99

B6 depletion or carcinoid syndrome may cause which clinical symptoms?

Back 99

Pellagra (diarrhea, dermatitis, mental changes); Niacin deficiency

Front 100

B6 deficiency

Back 100

Irritability, anemia, convulsions

Front 101

B5 deficiency

Back 101

Alopecia, dermatitis, enteritis

Front 102

B2 deficiency

Back 102

Cheilosis, corneal vascularization, angular stomatitis (FACE)

Front 103

B1 def

Back 103

Wernickes or Beri Beri (Wet = dilated CHF, dry = neuropathy, symmetrical muscle atrophy)

Front 104

Anything involved CoA also involves…

Back 104

B5 (pantotheone)

Front 105

VitC helps collagen, but also (2)

Back 105

Helps absorb Fe2+ and helps convert DA to NE

Front 106

VitE

Back 106

Antioxidant in RBCs

Front 107

Less hormone-induced hair, hypogonadism

Back 107

Zinc deficiency

Front 108

B6 uses

Back 108

Making heme, glycogen phosphorylase; plays a role in homocysteinuria (helps overcome enzyme affinity decrease)

Front 109

HS + CH3 --> _____, mediated by what

Back 109

Methionine, mediated by THF-CH3 + B12

Front 110

B12 deficiency causes neuropathy when what accumulates

Back 110

Met down or methylmalonyl-CoA up (can't enter Kreb's cycle at succinyl CoA)