Porphyrias: acute intermittent porphyria symptoms 5 P's:

Pain in abdomen

Polyneuropathy

Psychologial abnormalities

Pink urine

Precipitated by drugs (eg barbiturates, oral contraceptives, sulpha drugs)

 

BUN:creatinine elevation: causes ABCD:

Azotremia (pre-renal)

Bleeding (GI)

Catabolic status

Diet (high protein parenteral nutrition)

 

G6PD: oxidant drugs inducing hemolytic anemia AAA: Antibiotic (eg: sufamethoxazole)

Antimalarial (eg: primaquine)

Antipyretics (eg: acetanilid, but not aspirin or acetaminophen)

 

Vitamin B3 (niacin, nicotinic acid) deficiency: pellagra

The 3 D's of pellagra: Dermatitis Diarrhea Dementia

· Note vitamin B3 is the 3 D's.

 

Tangier's disease: hallmark

"Tangierene tonsils":

Hallmark is large orange tonsils.

· Important clinical note: there is no increased risk of atherosclerosis, just like eating tangerenes.

 

Na+/K+ pump: movement of ions and quantity

K+ and in each consist of 2 characters, so so 2 K+ are pumped in. Na+ and out each consist of 3 characters, so 3 Na+ are pumped out

 

Glycogen storage: Anderson's (IV) vs. Cori's (III) enzyme defect

ABCD:

Anderson's=Branching enzyme.

Cori's=Debranching enzyme.

· Otherwise, can't really distinguish clinically.

 

Nitric oxide: amino acid precursor

When the dentist works on your teeth, you say, "AAArg! (Arginine)" before he administers Nitric Oxide (NO) to take the pain away.

· Other players necessary for NO synthesis: NO synthase, Ca++, NADPH.

 

Pompe's disease: type "Police = Po + lys":

Pompe's disease is a lysosomal storage disease (alpha 1,4 glucosidase).

 

Metabolic acidosis (normal anion-gap): causes

· With hyperkalemia: RAISE K+:

RTA type 4

Aldosterone or mineralocorticord deficiency

Iatrogenic: NH4Cl, HCl

"Stenosis": obstructive uropathy

Early uremia

· With hypokalemia: ReDUCE K+:

Renal TA type 1 and 2

Diarrhoea

Urine diversion into gut

Carbonate anhydrase inhibitor

Ex-hyperventilation

 

Galactosaemia: enzyme deficiency

GALIPUT: Galactose 1 Phosphate Uridyl Transferase.

· There is an assay called the Galiput test for this.

 

Citric acid cycle compounds

"Our City Is Kept Safe And Sound From Malice":

Oxaloacetate Citrate Isocitrate alpha-Ketoglutarate Succinyl-CoA Succinate Fumarate Malate

 

Folate deficiency: causes A FOLIC DROP:

Alcoholism

Folic acid antagonists

Oral contraceptives

Low dietary intake

Infection with Giardia

Celiac sprue

Dilatin

Relative folate deficiency

Old

Pregnant

 

Mitochondrial DNA (mt DNA) properties

"mt DNA".

mt stands for: Maternal Transfer Mutates Tremendously (high mutation rate)

 

Vitamins: which are fat soluble

"The FAT cat is in the ADEK (attic)":

Fat soluble vitamins are A,D,E,K.

 

Enzyme kinetics: competitive vs. non-competitive inhibition

With Kompetitive inhibition: Km increases; no change in Vmax.

With Non-kompetitive inhibition: No change in Km; Vmax decreases.

 

Glycogen storage: names of types I through VI

"Viagra Pills Cause A Major Hardon":

Von Gierke's

Pompe's

Cori's

Anderson's

McArdle's

Her's

 

Sickle cell disease pathophysiology

SICKle cell disease is due to a Substitution of the SICKsth amino acid of the B chain.

 

Glucagon function

"Mr. Gluca has Gone to the cAMP to bring out some Glucose":

· Glucagon elevates glucose by cAMP mechanism.

 

Van den Bergh reaction (Jaundice test)

"Indirect reacting bilirubin = Unconjugated bilirubin":

Both start with vowels, so they go together:

Indirect & Unconjugated.

 

Hemoglobin binding curve: causes of shift to right

"CADET, face right!":

 CO2

Acid

2,3-DPG (aka 2,3 BPG)

Exercise

Temperature

 

G proteins for respective receptors

"QISS & QIQ" (Kiss and Kick):

· G-proteins and their respective receptors (alphabetical order):

Q: alpha 1 I: alpha 2 S: beta 1 S: beta 2 &

Q: M1 I: M2 Q: M3

 

Adrenaline mechanism

"ABC of Adrenaline":

Adrenaline--> activates

Beta receptors--> increases

Cyclic AMP

 

Citric acid cycle compounds

"Can I Keep Selling Sex For Money, Officer?":

Citrate Isocitrate alpha Ketogluterate Succinyl CoA Succinate Fumerate Malate Oxaloacetate

 

Carbon monoxide: electron transport chain target

"CO blocks CO":

Carbon monoxide (CO) blocks Cytochrome Oxidase (CO)

 

Metabolism sites

"Use both arms to HUG":

Heme synthesis Urea cycle Gluconeogenesis · These reactions occur in both cytoplasm and mitochondria.

 

Na/K pump: concentrations of Na vs. K on inside/outside of cell, pump action, number of molecules moved

HIKIN': There is a HIgh K concentration INside the cell. From this can deduce that the Na/K pump pumps K into cell and Na out of cell.

Alternatively: When I was learning this pump (circa 1992), a band that was "in" was Kris Kross, and a band that was "out" was "Sha Na Na Na": So pump moves K K (2 K) in and Na Na Na (3 Na) out. · Sadly, as infectious as their debut album was, Kris Kross can really no longer be classed as "in".

 

DNA bond strength (nucleotides)

"Crazy Glue":

Strongest bonds are between Cytosine and Guanine, strong like Crazy Glue (3 H-bonds), whereas the A=T only have 2 H-bonds. · This is relevant to DNA replication, as the weaker A=T will be the site where RNA primer makes the initial break.

 

Dicarboxylic acids (alpha, omega) C2 through C10: common names

"Oh My, Such Good Apple Pie, Sweet As Sugar!":

Oxalic Malonic Succinic Glutaric Adipic Pimelic Suberic Azelaic Sebacic

 

Infantile Beriberi symptoms

Restlessness Sleeplessness Breathlessness Soundlessness (aphonia) Eatlessness (anorexia) Great heartedness (dilated heart)

· Alternatively: Get 5 of 'em with BERI: Breathless/ Big hearted, Eatless, Restless, Insomnia.

 

 

AcetylCoA and acetacetylCoA: amino acids forming them

"A Lighter Lease" (A LyTr LeIs):

A=AcetylCoA or Acetoacetyl CoA

Ly=Lysine

Tr=Tryptophan

Le=Leucine

Is=Isoleucine

 

Fasting state: branched-chain amino acids used by skeletal muscles

"Muscles LIVe fast":

Leucine

Isoleucine

Valine

 

Glycolysis steps

"Goodness Gracious, Father Franklin Did Go By Picking Pumpkins (to) Prepare Pies":

Glucose

Glucose-6-P

Fructose-6-P

Fructose-1,6-diP

Dihydroxyacetone-P

Glyceraldehyde-P

1,3-Biphosphoglycerate

3-Phosphoglycerate

2-Phosphoglycerate (to)

Phosphoenolpyruvate [PEP] Pyruvate · 'Did', 'By' and 'Pies' tell you the first part of those three: di-, bi-, and py-.

· 'PrEPare' tells location of PEP in the process.

 

Fabry's disease

FABRY'S:

Foam cells found in glomeruli and tubules/ Febrile episodes

Alpha galactosidase A deficiency/ Angiokeratomas

Burning pain in extremities/ BUN increased in serum/ Boys

Renal failure

YX genotype (male, X linked recessive)

Sphingolipidoses

 

B vitamin names

"The Rhythm Nearly Proved Contagious":

· In increasing order: Thiamine (B1) Riboflavin (B2) Niacin (B3) Pyridoxine (B6) Cobalamin (B12)

 

Electron transport chain: Rotenone's site of action

Rotenone is a site specific inhibitor of complex one.

 

Citric acid cycle compounds

"Oh! Can I Keep Some Succinate For Myself?":

Oxaloacetate Citrate Isocitrate Ketoglutarate Succinyl coA Succinate Fumarate Malate

 

Citric acid cycle compounds

"Oh Citric Acid Is Of (course) A SiLly STupid Funny Molecule":

Oxaloacetate Citrate Aconitate Isocitrate Oxalosuccinate Alpha-ketoglutarate SuccinyL-CoA SuccinaTe Fumarate Malate

· SilLy and sTupid used to differentiate succinyL and succinaTe

 

Type 1 glycogen storage disease

Type 1 = one (Von), ie Von Giereke's disease

 

Essential amino acids

PVT. TIM HALL: Phe Val Thr Trp Ile Met His Arg Leu Lys

· Pvt. is short for Private in the military.

· Arg and His are considered semiessential.

· Alternatively: "MATT VIL PHLy".

 

Citric acid cycle compounds

"Can I Ask Sharon Stone For My Orgasm?":

Citrate Isocitrate Alpha-Ketoglutarate Succinyl CoA Succinate Fumerate Maleate Oxaloacetate

 

Golgi complex: functions

"Golgi Distributes A SPAM":

Distributes proteins and lipids from ER

Add mannose onto specific lysosome proteins

Sulfation of sugars and slected tyrosine

Proteoglycan assembly

Add O-oligosugars to serine and threnonine

Modify N-ologosugars on asparagine

 

Pyruvate: products of complete oxidation

"4 Naked Fun 3 Coeds + 1 Guy":

· Complete oxidation of pyruvate yields:

4 NADH FADH2 3 CO2 1 GTP

 

Catabolism steps of branched chain amino acids

"Truck hit the Ox to Death":

Transamination

Oxidative decarboxylation

Dehydrogenation

 

Niacin deficiency: signs and symptoms

The famous 4 D's:

Diarrhoea Dermatitis Dementia Death (if untreated)

 

Creatine phosphate: amino acid precursors

"Nice GAMs!": Glycine Arginine Methionine

· Gam is slang for a person's leg, especiallay an attractive female's leg: "Nice gams = nice legs!"

· Creatine phosphate is a muscle energy store, and spontaneously converts to creatinine which is excreted in the urine in direct proportion to muscle mass: clinically useful, such as in MS Dx.

 

Enzymes: classification

"Over The HILL":

Oxidoreductases

Transferases

Hydrolases

Isomerases

Ligases

Lyases

· Enzymes get reaction over the hill.

 

Insulin: function

INsulIN stimulates 2 things to go  IN 2 cells: Potassium and Glucose.

 

Collagen concisely covered

COLLAGEN:

C-terminal propeptide (procollagen)/ Covalent Cross links/ C vitamin/ Connective tissue/ Cartilage/ Chondroblasts/ Copper Cofactor (Covalent Cross linking)

Outside the cell is where collagen normally functions/ Osteoblasts/ Osteogenesis imperfecta

Lysyl hydroxylase/ Lysyl oxidase (oxidatively deaminates lysyl and hydroxylysyl residues to form collagen cross links, last biosynthesis step)

Long triple helical fibers/ Ligaments

Alpha chains/ Attached by H bonds form triple helix/ Ascorbate for hydroxylation of lysyl and prolyl residues of pro-Alpha chains (postranslational modification)

Gly in every third position/ Glycosylation of hydroxyl group of hydroxylysine with Glucose and Galactose; GOlgi allows procollagen to GO outside of cell

Extracellular matrix/ Eye (cornea, sclera)/ Ehlers-Danlos Syndrome

N-terminal propeptide (procollagen)/ Nonhelical terminal extensions

· Note: Procollagen LEAVEs the cell to be cLEAVEd by procollagen peptidases

 

Essential amino acids

"PVT. TIM HALL always argues, never tires":

Phe Val Thr Trp Ile Met His Arg Lue Lys

· Always argues: the A is for Arg, not Asp.

· 'Never tires': T is not Tyr, but is both Thr and Trp.

 

Hypervitaminosis A: signs and symptoms

"Increased Vitamin A makes you HARD":

Headache/ Hepatomegaly

Anorexia/ Alopecia

Really painful bones

Dry skin/ Drowsiness

 

Heme synthesis: amino acid precursors to basic unit of porphyrins, heme (pyrrole ring)

"Dracula wants to Suck a Co-ed's blood [think heme] with his Glystening teeth!":

Succinyl CoA and Glycine are precursor amino acids to pyrrole rings, which is the basic unit of porphyrins and heme.

 

Enzymes: competitive inhibitors

"Competition is hard because we have to travel more kilometers (Km) with the same velocity":

With competitive inhibitors, velocity remains same but Km increases

 

Phosphorylation cascade: action during low glucose

"In the Phasted State, Phosphorylate":

The phosphorylation cascade becomes active when blood glucose is low.

 

Sickle cell anemia: mutation

"HbS isn't Very Good":

At Sixth position of HB beta chain, Valine is present instead of Glutamic acid.

 

tRNA: molecular shape

Fortunately, tRNA (Transfer RNA) is shaped like a capital T.

· See diagram.  

 

Malate-aspartate shuttle

"MAD commute":

Malate in. Alpha-ketoglutarate and D (Aspartate) out.

 

Vitamins: which are fat soluble

KADE: Vitamin K Vitamin A Vitamin D Vitamin E

 

Phenylketonuria: which enzyme is deficient

PHenylketonuria is caused by a deficiency of:  Phenylalanine Hydroxylase

 

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