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Biochemistry SJSM > Block 3 GC > Flashcards

Block 3 GC Flashcards

1
Q

Heme synthesis pathway
“GLASs PHotos of Ugly Corgis, Poodles, & Hounds”

A

Mito:
—> Glycine + Succinyl-CoA combine to make Aminolevulinic acid (via ALA synthase+ B6 (biotin))

Cyto:
—> Aminolevulinic acid leaves the mito & forms Porphobilinogen (via ALA Dehydrase)
—> Porphobilinogen forms Hydroxymethylbilane (via Porphobilinogen Deaminase/Hydroxymethylbilane synthase)
—> Hydroxymethylbilane forms Uroporphyrinogen III (via Uropophrynogen synthase)
—> Uroporphyrinogen forms Coporhyrinogen III (via Uroporphyrinogen Decarboxylase)

Mito:
—> Corporphyrinogen forms Protoporphyrin IX
—> Protoporphyrin forms Heme (via Ferrochetalase + Fe2+)
‘

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2
Q

Inhibition of Heme synthesis

  • Lead (affects which 2 enzymes)
    &
    Heme & high glucose
    Low iron
A

Lead: inhibits ALA Dehydrase & Ferrochetalase

Heme/hematin/hemenin & high glucose: ALA synthase I (Liver)

Low iron: ALA synthase II (Bone marrow)

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3
Q

Heme synthesis disorders:

Acute intermittent porphyria
“Ads No ACcePtABLe Pain”

A

Deficient Porphobilinogen Deaminase/Hydroxymethylbilane synthase:

Auto Dom + Late onset

Causes build-up of PBG (Porphobilinogen) & ALA (Cause neurological dysfunction)
Symptoms:
- Anxiety, confusion, Paranoia
- Acute Abdominal pain
- Port-wine urine (sometimes)

DON’T GIVE BARBITUATES or fast

5 P’s:
- Painful abdomen
- Port-wine urine
- Polyneuropathy
- Psych issues
- Precipitated by barbituates

Treat:
- Hematin/hemin
- high CHO diet

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4
Q

Heme synthesis disorders:

Porphyria cutanea tarda
“Ads Heat, PAin, & BuRns”

A

Deficient Uroporphyrinogen Decarboxylase

Auto DOM + Late onset

Causes build-up of cyclic tetrapyrroles (cause sunlight-induced lesions)

Symptoms:
- Photosensitivity
- Blistering/inflammation of the skin
- Red/Brown urine (build-up of uroporphyrinogen & abnormal liver enzymes)
- Hyperpigmentation

WORSE WITH ALCOHOL

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5
Q

Port-wine urine is due to build-up of which substrate & is associated with what conditions?

A

Build-up: Uroporphyrinogen

Ass.:
- Hepatitis
- HIV
- Alcoholism
- Oral contraceptives
- Acute int porphyria
- Porphyria cutanea tarda

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6
Q

Anemia types:

Microcytic (less than 80)
“MIcro AnTS”

A
  • Iron deficiency
  • ACD
  • Thalassemias
  • Sideroblastic anemia
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7
Q

Anemia types:

MCV (80-100)
Normocytic-Hemolytic & Intrinsic anemia

“Normal HIGHSchool Pressure”

A
  • G6PD, PK
  • Spherocytosis (hereditary
  • Paroxysmal nocturnal hemoglobinuria
  • Sickle cell
  • Hbc
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8
Q

Anemia types:

MCV (80-100)
Normocytic-Hemolytic & Extrinsic anemia

“A NormAl HEMIsphere”

A
  • MAHA
  • MIHA
  • Auto immune
  • Infection
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9
Q

Anemia types:

MVC (80-100)
Normocytic-Nonhemolytic anemia

“NAKed Ass”

A
  • ACD
  • Kidney disease
  • Aplastic anemia
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10
Q

Anemia types:

Macrocytic-Megaloblastic anemia

“Massive Mega-Bull Frogs”

A
  • Megaloblastic
  • B12 Def
  • Folate def
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11
Q

Anemia types:

Macrocytic-Megaloblastic: B12 def absorbed in the ileum
“VitAMin B12 in Breakfast Cereal, HeMP, Trout, HeARTy AniMal Meats, & PeAs”

A
  • Risks (Vegans, Atrophic gastritis, Bypass surgery, Tapeworms, ***Percarnious anemia, Crohn’s, AIDS)
  • 2-3 yrs Onset
  • High methylmalonic acid
  • Megaloblastosis
  • Peripheral Neuropathy
  • Affected MM-CoA & Homocysteine methyltransferase
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12
Q

Anemia types:

Macrocytic-Megaloblastic: Folate def
“FolATe Affects How PregnanT woMen Deliver”

A
  • risks (Preggos, ***Alcoholics, & Drug users (phenytoin & spruce)
  • THF (active form via DHFR)
  • Affects Thymidylate synthase, Homocysteine methyltransferase, & purine synthesis
  • Megaloblastosis (ONLY)
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13
Q

Microlytic anemia: MCV < 80

Iron Deficiency
“IroN BinDs 2-3x Less THen TItanium Without PacKinG”

A

Iron def:
- Normal ALA

-Bleeding (colon cancer, GI ulcer, Menorrhagia
etc.)

  • Diet def (Protein def, ascorbic acid, vit C, or
    copper)
  • Low Ferritin (Fe3+) & Iron (Fe2+) in serum
  • Tachycardia, Weakness/parllor, Glossitis, Pica, Koilonychia (spooning nails)
  • High TIBC (body wants more iron)
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14
Q

Microcytic anemia:

Sideroblastic anemia
- B6 deficiency
“IOns LAST & RADiatE Colour in Space”

&

  • Lead toxicity
    “LABS PrOvIde bLADes For iNcisions”
A

B6 Def:
- Isoniazid (TB patients)
- Low ALA synthase (Low ALA levels mean no
Heme to bind iron)

  • Iron overload (high ferritin & iron)
  • Low TIBC (the body doesn’t need more iron)
  • Reduced AA decarboxylase activity (def GABA,
    DA, NE/E = Epileptic convulsions)
  • Reduced CBS activity (Homocystinemia & risk
    DVT/Stroke)

Lead poisoning:
- Lead exposure (paint chips)
- Abdominal pain (unexplained)
- Basophilic Stippling
- Peripheral neuropathy
- Iron Overload (high ferritin & iron)
- Low ALA Dehydrase (High ALA levels)
- Low ferrochelatase (sideroblastosis)
- Low TIBC (the body doesn’t want more iron)
- Neuro-toxic/psychic issues

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15
Q

Microcytic anemia:

Thalassemia
“thAlAssemiA Has Bad CHanges”

A

Alpha thalassemia:
- 1 deletion = asymptomatic
- 2 deletions = Mild anemia
- 3 deletions = Severe anemia
- 4 deletions = Hydropis fetalis (dead)

Beta thalassemia:
- 1 deletion = Minor B-T
- 2 deletions = Major B-T
(Hgb A2) reduces hematopoiesis in normal bone marrow in long bones, so it happens more in the skull (Chimpmunk Facies)

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16
Q

Microcytic/Normocytic anemia:

ACD (Anemia of Chronic Diseases)
“CD’s in the KAR”

A

The body can’t tell the difference between chronic inflammation and infection, so it hides its iron from siderophoric bacteria.

It releases Hepcidin (lowers iron reabsorption & increases storage)

Low iron & ferritin
High TIBC

Kidney disease
Autoimmune disease
Rheumatological disease

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17
Q

Normocytic anemia (80-100):

Paroxysmal Nocturnal Hemoglobinuria
“PNH Can DRoP PIGA”

A

Pancytopenia, Negative Coombes test, & Hibernation (during sleep)

  • Complement system isn’t inhibited by CD55/59
  • Defective PIGA gene
  • Decreased CD55/59
  • Premature RBC lyses
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18
Q

Normocytic Intrinsic anemia:
“NO FRee SHots of Gin or FireBall Pay your Dollars”

A
  • No NADPH
  • Glutathione isn’t reduced
  • Oxidative stress on RBCs
  • Free radicals aren’t destroyed
  • Sulfa, Dapsone, primaquine, & favabeans
  • Heinz bodies + Bite cells
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19
Q

Normocytic Intrinsic anemia:

Hereditary Spherocytosis
“DABS are Cool as SHIt”

A
  • Dsyfunctional RBC membrane protein (Ankyrin, Bands & Spectrin)
  • Coombs negative test
  • Splenomegaly
  • Howell-Jolly bodies
  • Indirect Jaundice
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20
Q

Key points of transamination:
“Don’T FRee Pesky NH3”

A
  • its a Double-reaction
  • Transaminase (aka aminotransferase) is the enzyme
  • it’s Freely Reversible
  • PLP (aka B6 derivative is the coenzyme)
  • No free Ammonia is liberated

ex.
Alanine aminoTR (ALT)
Serum Glutamate-Pyruvate Transaminase (SGPT)
(Alanine + a-ketoglutarate–>pyruvate + glutamate)

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21
Q

Why is Glutamate so important in the transanimation/urea cycle?

A

Because it’s the only AA that undergoes oxidative deamination enough to release ammonia to feed the urea cycle

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22
Q

Key points about oxidative deamination:

A
  • Glutamate Dehydrogenase (enzyme)
  • Liver mitochondria have GDH
  • NAD/NADP (coenzymes)
  • Releases NH3 to urea cycle
  • Allosterically inhibited (ATP, GTP, NADH)
  • Activated (ADP)
  • Reversible
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23
Q

AA degradation:

Phenylalanine

A

Phenylalanine -(BH4)–>
Tyrosine -(BH4)–> (also makes thyroxine)
DOPA -(B6)–> (also makes melanin)
Dopamine -(Vit C)–>
Norepi -(SAM)–>
Epi

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24
Q

AA Degradation:

Glutamate

A

Glutamate -(B6)–>
GABA & Glutathione

25
Q

AA Degradation:

Histidine

A

Histidine -(B6)–>
Histamine

26
Q

AA Degradation:

Tryptophan

A

Tryptophan
-(B2/6)–>
Niacin —> NAD+/NADH
&
-(BH4/6)–>
Serotonin —> Melatonin

27
Q

AA Degradation:

Glycine

A

Glycine -(B6)–>
Porphyrin —>
Heme

28
Q

AA Degradation:

Glutamate

A

Glutamate -(B6)–> GABA & Glutathione

29
Q

AA Degradation:

Arginine

A

Arginine -(BH4)–> Nitric oxide, urea, & creatine

30
Q

Cofactors for synthesis & degradation of AA:

B6

B9 (folic acid)

GTP

A

Pyridoxal phosphate (PLP)

Tetrahydrofolate (Fh4)

Tetrahydrobiopterin (BH4)

31
Q

Melanin Synthesis:

A

—> Tyrosine turns into Dihydroxy phenylalanine (DOPA) (via Tyrosinase & Cu2+)

—> Dihydroxy phenylalanine turns into DOPA Quinone (via Tyrosinase & Cu2+)

—> DOPA Quinone can turn into either Eumelanin (black polymer) spontaneously or Phenomelanin (yellow/red polymer)

Tyrosinase:
- Rate limiting
- Monooxygenase
- Cu cofactor

32
Q

Synthesis of Catecholamines

A

Happens in the chromaffin cells of the adrenal medulla:

33
Q

Purine Salvage pathway conditions”

Lesch-Nyhan syndrome
“eXtRA nh3 Can IMPact HGPRT’S tRansFerase”

A
  • X-linked REC
  • Allopurinol (1st Rx)
  • Choreoatheotic movements (slow+sudden movements)
  • Intellectual disability
  • Monosodium urate orange birefringent crystals
  • low Purine diet
  • Hyperuricemia (high uric acid aka gout)
  • Gouty arthritis
  • Spastic cerebral palsy
  • Rage (self-mutilation hands/lips)
  • Febuxostat (2nd)
34
Q

Purine Salvage pathway conditions

ADA deficiency syndrome
“ADa DRains T/B cellS(CID)”

A
  • Def Adenosine Deaminase
  • dATP inhibits Ribonucleotide Reductase (No DNA synthesis)
  • Low T/B cells + Ig’s
  • SCID (Severe Combined Immuno Deficiency)
35
Q

Mech of action of allopurinol

A

It inhibits xanthine oxidase to reduce uric acid production (common gout treatment & Lesch Nyhan synd treatment)

36
Q

Gout treatments

Acute case

Chronic Case

A

Acute: Colchicine (anti-inflammatory)

Chronic: Allopurinol + probenecid (decrease uric acid production via inhibiting xanthine oxidase & PRPP amidoTR)

37
Q

Gout (soft tissue, joints, & kidney stones)

Primary “Primary SHAG”

vs

Secondary “Lazy Loggers TRaiLeD After Cooked GrAvy”

A

Primary: Too much purine synthesis
- Superactive PRPP synthase (X-linked)
- Superactive PRPP amidoTR (Lesh-Nyhan)
- G-6-P def (type 1 glycogenesis def)

Secondary: too much uric acid
- Leukemia/lymphoma (too much prod)
- Renal failure, Thiazide, Lactic acidosis, Diuretics (reduced excretion)
- Acute Gouty Arthritis (metatarsal of the big toe)
- Chronic Gouty Arthritis (tophi deposits of monosodium urate crystals in tissue/joints

38
Q

Urea cycle disorders:

“ARginosuCcinate synthASe”

A
  • Auto REC
  • Citrullinemia
  • Mutated ASS1
39
Q

Urea cycle disorders:

CPS-1 def
“CPS-1 LOCks Free Ammonia”

A
  • Def Carbamoyl Phosphate synthase
  • Low citrulline
  • Ortic aciduria (high ortic acid)
  • Ammonia (hyperammonemia)
  • Feeding (poor reaction to 1st postnatal feed)
40
Q

Urea cycle disorders:

OTC def
“OTC eXAGgeRates hOW Low Citrulline isn’t”

A
  • Def Ornithine Transcarbamylase
  • X-linked REC
  • High Ammonia, Glutamine, & Ornithine
  • White crystals in urine
  • Low citrulline
41
Q

Pyrimidine pathway disorder:

UMP Synthase def
“uMp Synthase WOn’t BAN TUCkeRed Hot Lesbians”

A
  • Def Ump synthase
  • Severe chronic anemia
  • White needle-like crystals in urine
  • Ortic aciduria
  • Normal Ammonia & BUN
  • Treat via cytosine & uracil replacement therapy
  • Low Hb (~5g/gL)
42
Q

Anti Cancer Drugs:

“MethotRexAte CLoSes & ALMost DRIPS”

A
  • Rheumatoid arthritis
  • Abortion/ectopic preggos
  • Choriocarcinoma
  • Lymphoma
  • Sarcoma
  • Acute Lymphocytic Myelogenous Leukemia
  • Inhibited Dihydrofolate Reductase
  • S Phase
43
Q

Anti Cancer Drugs:

5’ Fluorouracil
“SiP IPA BITCheS”

A
  • S Phase
  • Inhibits Pyrimidine Analog
  • Basal cell carcinoma
  • Inhibits Thymidylate synthase (reduce dTMP & DNA synthesis)
  • Colon cancer
  • Solid tumors
44
Q

Anti-Cancer drugs:

Hydroxyurea
“SPIRR MCS”

A
  • S Phase
  • Inhibits Ribonucleotide Reductase
  • Melanoma
  • CML
  • Sickle Cell
45
Q

Carcinoid Tumors:

“cArcinoiDS Produce Gross HIAtAl”

A
  • Amine Precursors Uptake Decarboxylase Cells
  • Diarrhea
  • High Serotonin & Sweating
  • Pellagra Niacin def (B3) (Diarrhea, Dementia, Dermatitis)
  • Gi Tumors
  • 5-HIAA (5-Hydroyindoleacetic acid)
46
Q

Hartnup Disorders
“hARTNUP CAuses problems”

A
  • Auto REC
  • Tryptophan absorption deficiency (Renal + Intestine)
  • High Neutral AA
  • Unsteady wide gait
  • Pellagra (Dementia, Dermatitis, Diarrhea)
  • Cutaneous photosensitivity
  • Ataxia
47
Q

Catecholamine synthesis disorders:

Albinism Turns white

A

Def Tyrosinase
less melanin

48
Q

Catecholamine synthesis disorders:

“alkAPTONUria Hurts”

A
  • Arthritis
  • Treat via low Phenylalanine & Tyrosine diet
  • Orchronosis
49
Q

Catecholamine synthesis disorders:

Phenylketonuria
“PHEnylKEtonuria SAPs My energy”

A
  • Phenylalanine Hydroxylase deficiency
  • Eczema
  • Phenyl ketones in urine
  • Epilepsy
  • Short
  • Avoid Phenylalanine (Aspartame)
  • Musty odor
50
Q

MCAD (Med-chain Acyl-Coa Dehydrogenase def)
“HARty HELMinths HaVe Harsh CLaws”

A

Hyperammonemia
Auto REC
Hypoketotic
Elevated Liver enzymes
Metabolic acidosis
Hyperuricemia
Vomit
Hypoglycemia
Coma
Lethargy

51
Q

Primary Carnitine def
Higher risk in
- Vegans, liver/kidney failure
“CARnititine def is HarmFuL, HarsH, & HELlishly DestructiVe”

A

Coma
Auto Rec
Hypoketotic Hypoglycemic
Fail to thrive
Hyperammonemia
Hypotonic
Elevated Liver enzymes
Death
Vomit

52
Q

Carnitine Palmitoyl transferase def 2 (CPT2)
“CARNitiNe 2 def is HarsH, HarMFul, & HELlisH”

A

Eat CARBS!
Auto REC
Neonatal
Hypoketonia
Hypoglycemia
Hyperammonemia
Myalgia
Fail to thrive
Hypotonia
Elevated Liver enzymes
High/normal carnitine levels

53
Q

Which organs use what fuel source in the Fed, Fasted, & Straved states?

Brain
Heart
Muscles
Liver
RBCs

A

Brain:
- Fed (glucose)
- Fast (glucose)
- Starve (ketones)

Heart:
- Fed (fatty acids)
- Fast (fatty acids)
- Starve (ketones)

Muscles:
- Fed (glucose)
- Fast (fatty acids)
- Starve (fatty acids/ketones)

Liver:
- Fed (glucose)
- Fast (fatty acids)
- Starve (amino acids)

RBCs:
- Fed (glucose)
- Fast (glucose)
- Starve (glucose)

54
Q

Ketone vs cholesterol synthesis

Location
Intermediate

A

Ketone:
Mitochondria
HMG-CoA Synthase

Cholesterol:
Cytoplasm/sER
HMG-CoA Synthase

55
Q

Cholesterol synthesis steps
“All Areas Have Many Interesting Fashion Styles & Lacy Corsets”

A

2X Acetyl-CoA
(Thiolase)
Acetoacetyl-CoA
(HMG-CoA Synthase)
HMG-CoA
(HMG-CoA Reductase RLE)
Mevalonate
Isoprenoid
Farnesyl
Squalene
Lanesterol
Cholesterol

56
Q

Competitive inhibitor of Cholesterol synthesis are

57
Q

HMG-CoA Reductase is inhibited via which two products of cholesterol synthesis?

A

Mevalonate & Cholesterol

58
Q

HMG-CoA Reductase is activated via which two hormones?

A

Insulin & Thyroxine

59
Q

HMG-CoA Reductase is inactivated via which two hormones?

A

Cortisol & glucagon

Biochemistry SJSM (9 decks)

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