Biochem smart book Flashcards

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1
Q

Where does the phosphorylated mannose get trasnported to from the Golgi?

A

lysosomes

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2
Q

Describe Glycogenolysis in the muscle and liver with Glucagon epinephrine and what they act on

A

In the muscle it starts with muscle contraction. this works via Ca+2 via phosphorylase kinase

muscle contraction –>Ca–> phosphorylase kinase

Muscle also has Epi receptors that work through cAMP –> Protien Kinase A –> glycogen phosphorylase kinase

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3
Q

What all is Collagen made of? most abundant amino acid?

A

Collagen is made up of alpha chains that form triple helix via hydrogen bonds

Glycine most abundant

followed by luecine and proline

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4
Q

which enzyme has exonuclease and whats the function of that enzyme?

A

DNA polymerase III and it synthesizes DNA along with proofreading for mismatches in the gena

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5
Q

If a person is acidotic, due to increased NADH which enzyme is increased / decreased

A

Lactate dehydrog is increased (lactic acidosis)

____

Pyruvate to Acetyl coa is decreased (nadh means you dont have many dehydrogenase reactions going on)

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6
Q

Whats wrong in HbS?

A

Sickle cell anemia

Glutamine–> Valine

allows hydrophobic reaction among the Hb which leads to sickling

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7
Q

Which enzyme turns NE to Epi? what is this stimulated by?

A

PNMT -Phenylethanlolamine-N-Methyltransferase

Cortisol stimulates this

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8
Q

What is heme first metabolized to? whats the enzyme? and why does it turn Green?

A

Heme —-Heme oxygenase—>Billeverdine (green)

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9
Q

what causes Right shifts in the O2 dissociation curve?

A

incr. H+/ 2,3 BPG / Temp(excercise)

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10
Q

What amino acid is important for maximizing acid secretion in the urine?

A

Glutamine

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11
Q

How long does it take for B12 stores to deplete?

how do you geet b12 deficiency

A

4-5 years

Vegan for a long time / Gastrectomy for a long time (pernicious anemia) / Diphthoryloborium (tapeworm)

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12
Q

Insulin activates _____ to stimulate glycologen synthesis

A

Protein Phosphatase

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13
Q

what are the stop codons?

A

(you go away)

UGA

UAA

UAG

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14
Q

Whats the function of phospholipase C, IP3

A

PLC cleaves PIP2 into Dag & IG3

IP3 opens sarcoplasmic reticulum to release Ca+ which activates Phosphokinase C

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15
Q

in regards to maintainng glucose, what helps pyruvate fit into gluconeogenesis. whats a necesary cofactor?

A

Pyruvate has to be turned into Oxoaloacetate first

Pyruvate carboxylase

the cofactor is Acetyl Co A

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16
Q

when heme binds oxygen, it must first release ____ and _____ which then get converted to ______

A

H+ and CO2

gets converted to bicarb

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17
Q

whenever you get high oxygen affinity (left shift) hemeglobin what is the sequela that follows?

A

reduced ability to deliver O2—> hypoxia —> EPO synthesis —> Erythrocytosis

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18
Q

Which amino acid is responsible for transfering nitrogenous waste to the liver?

in the liver what amino acid is responsible for disposal of nitrogenous waste?

what enzyme is responsible for turning alanine into glucose vice versa

A

Alanine

Glutamate

A-ketoglutarate

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19
Q

baby cant sit up w/o support, cant hold head up, neuronal regression (loss of social smile), cherry red macula, hepatosplenomegaly

A

Nieman pick dz

Sphingomyelinase deficiency

(not to be confused with Tay sachs = no hepatosplenomegaly)

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20
Q

smells like burnt sugar

diet restriction and treatment?

A

Maple syrup dz

I Love Vermont maple syrup from tree B1anches

Isoleucine, Luecine, Valine

supplement with B1 (Thiamine)

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21
Q

what is sucrose made of?

A

Fructose and glucose

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22
Q

whats the substitution for sickle cell?

A

Glu -> Val

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23
Q

Where does the amino acid bind to on tRNA

A

the CCA tail

tRNA looks like a clover leaf

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24
Q

Pee turns black after being left out for a while.

whats the dz, whats the deficiency, enzyme, accumulation

A

Alkaptonuria

cant turn

Tyrosine –>Homogentisate—HAD–> Fumarate

homogentisic acid dehydrogenase

accumulation of homogentisate

25
Q

function of SER

A

synthesis of Steroids and cholesterol

detoxification

26
Q

G coupled protein receptor, what part makes up the anchoring system

A

Transmembrane domain

non polar, hydrophobic amino acids

alpha helical

27
Q

does cortisol work in the cytoplasm or surface membrane?

A

cytoplasm

28
Q

What are the functions of NADPH

A

Reduce (good) Glutathione

Fatty acid, steroid, cholesterol Synthesis

29
Q

Whats Acute intermitent Porphyria

how do you treat it?

A

Its a fuck up of Heme synthesis

associated with PBG deaminase /

ALA synthase *rate limiting*

tx by inhibiting ALA synthase via heme or glucose

5 ps: Painful abdomen, Port wine pee, psych probs, polyneuropathy, precipated by drugs, alc, starvation

30
Q

What are zinc fingers? where are they and what do they bind to?

A

These are in the ctyoplasm that bind to the DNA for transcription

Steroids (estrogen, aldosterone, cortisol), thyroid hormone, fat soluble vitamins

31
Q

Where does insulin act on? what receptor and what tissue?

A

GLUT 4

Skeletal Muscle and adipocytes

32
Q

what enzyme is fucked up in lead poisoning

A

ALA Dehydratase

Ferrochetalase

33
Q

whats a cofactor of the reaction that turns Phenylalanine into Tyrosine

A

Phenylalaine —Phenylalanine hydroxylase–>tyrosine

it uses BH4 / Dihydrobiopterin

34
Q

defect in B- oxidation (fat degredation)

where does it occur/ whats the enzyme/ lab findings

A

Fat degredation (B-oxidation) occurs in the mitochondria

the enzyme is Acy-CoA dehydrogenase

Hypoketonic hypoglycemia

35
Q

what kind of anemia is stained prussian blue? which drug can cause this anemia?

A

SIderoblastic anemia

Isoniazid due to B6 (pyridoxine) deficiency

36
Q

What are the types of hemoglobin

A
37
Q

What does sorbitol get broken down to in healthy individuals?

whats the reaction and enzymes

A

Fructose

glucose–Aldose reductase-> Sorbitol –sorbitol dehydrogenase–> Fructose

38
Q

what does galactosemia predispose to?

A

E. coli infections

39
Q

where does microRNA/Small interfereingRNA act on? and what do they do?

A

These are used to induce Posttranscriptional Silencing of genes

mRNA

40
Q

What causes lactic acidosis

whats the primary site of lactate clearance?

A

it can be a sequela of septic shock due to hypoxia which impairs OX-phos causing a shunting of pyruvate to lactate

-Liver

41
Q

whats the enzyme after g6pd?

turns Ribose-5-p to Fructose-6-p

A

Transketolase

42
Q

Which vitamin gets deficient when you eat a bunch of raw eggs?

what reactions does this fuck up

A

B7 Biotin

Carboxylation

43
Q

Fructose 2,6 bisphosphonate

A

Insuline increases F26B which inihibits glycolysis

Glucagon decreases F26B which stimulates gluconeogensis

44
Q

What do RAS proteins bind to?

A

GTP

45
Q

What is the process where a single gene is recombined in different ways that produce different proteins?

A

Alternate RNA Splicing

46
Q

Whats the deficiency in Pompe dz

what findings will you see

A

its a Glycogen storage dz

A-glucosidase

Pompe trashes the pump (Cardiomegaly)

Glycogen in lysosomes

47
Q

What are the reactions that occur in the cytoplasm

A

HMP (pentose phosphate)

Glycolysis

Fatty acid Synthesis

48
Q

Whats special about Primase

A

its a DNA dependent RNA Polymerase

capable of making Uracil

49
Q

Where is ketone metabolism and which tissue cant use them

A

Mitochondria

Erythrocytes

50
Q

how do erythrocytes get their energy since they dont have mitochondria

A

Via glycolysis but they have to make 2,3BPG

51
Q

which enzyme neutralizes free radicals in cells

A

Superoxide dismutase

52
Q

Whats the Wobble hypothesis

A

This says how tRNA can bind to two different codons due to the 3rd letter haveing wiggle room.

ie. 2 separate codons can code for the same protien

CUC / CUU

because the first two letters are the same. the last has wobble room

53
Q

Which enzyme is responsible for moving short fragments of RNA primer? how does it read?

A

DNA polymerase I

5-3 exonuclease

54
Q

Whats ristocetin and how do you use it to differentiate between vWF dz and Bernard souiller

A

Ristocetin measure vWF because it activates the Glycoproten Ib (GP Ib) making it available to bind to vWF

two part test (in a patien with vWF deficiency)

1) so if you add ristocetin and it doesnt aggregate
2) add in normal plasma to sample and it does aggregate

______

in a patient with Bernard soullier

1) add ristocetin and doesnt aggregate
2) addition of normal plasma doesnt aggregate

55
Q

which substance inhibits B-oxidation in a well fed state

A

Malonyl-Coa

56
Q

Which ezyme helps breakdown fructose when you have a fructokinase deficieny

A

Hexokinase

57
Q

whats the difference between Germline mosacism and somatic?

A

Germline mosaicims means 2 normal parents give birth to affected kids

________

Somatic mosaicism means an affected parent has normal kids

58
Q

What is a luecine zipper?

A

2 alpha helical

Luecine in every 7th position

its a TRANSCRIPTION FACTOR

59
Q

What are the dz associated with VHL

A

RCC, hemangioblastoma, Pheo