Biochem smart book Flashcards by Trevor Johnson

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

lysosomes

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

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

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

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

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

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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Whats wrong in HbS?

Sickle cell anemia

Glutamine–> Valine

allows hydrophobic reaction among the Hb which leads to sickling

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

PNMT -Phenylethanlolamine-N-Methyltransferase

Cortisol stimulates this

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

Heme —-Heme oxygenase—>Billeverdine (green)

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what causes Right shifts in the O2 dissociation curve?

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

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

Glutamine

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How long does it take for B12 stores to deplete?

how do you geet b12 deficiency

4-5 years

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

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Insulin activates _____ to stimulate glycologen synthesis

Protein Phosphatase

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what are the stop codons?

(you go away)

UGA

UAA

UAG

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Whats the function of phospholipase C, IP3

PLC cleaves PIP2 into Dag & IG3

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

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

Pyruvate has to be turned into Oxoaloacetate first

Pyruvate carboxylase

the cofactor is Acetyl Co A

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

H+ and CO2

gets converted to bicarb

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

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

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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

Alanine

Glutamate

A-ketoglutarate

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

Nieman pick dz

Sphingomyelinase deficiency

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

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smells like burnt sugar

diet restriction and treatment?

Maple syrup dz

I Love Vermont maple syrup from tree B1anches

Isoleucine, Luecine, Valine

supplement with B1 (Thiamine)

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what is sucrose made of?

Fructose and glucose

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whats the substitution for sickle cell?

Glu -> Val

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Where does the amino acid bind to on tRNA

the CCA tail

tRNA looks like a clover leaf

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Pee turns black after being left out for a while.

whats the dz, whats the deficiency, enzyme, accumulation

Alkaptonuria

cant turn

Tyrosine –>Homogentisate—HAD–> Fumarate

homogentisic acid dehydrogenase

accumulation of homogentisate

function of SER

synthesis of Steroids and cholesterol detoxification

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

**Transmembrane domain** non polar, hydrophobic amino acids **alpha helical**

does cortisol work in the cytoplasm or surface membrane?

cytoplasm

What are the functions of NADPH

Reduce (good) **Glutathione** Fatty acid, steroid, cholesterol **Synthesis**

Whats Acute intermitent Porphyria how do you treat it?

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

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

These are in the **ctyoplasm** that bind to the DNA for transcription Steroids (estrogen, aldosterone, cortisol), thyroid hormone, fat soluble vitamins

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

GLUT 4 Skeletal Muscle and adipocytes

what enzyme is fucked up in lead poisoning

ALA Dehydratase Ferrochetalase

whats a cofactor of the reaction that turns Phenylalanine into Tyrosine

Phenylalaine ---*Phenylalanine hydroxylase*--\>tyrosine it uses **BH4** / **Dihydrobiopterin**

defect in **B- oxidation** (fat degredation) where does it occur/ whats the enzyme/ lab findings

Fat degredation (*B-oxidation)* occurs in the **mitochondria** the enzyme is **Acy-CoA dehydrogenase** **Hypoketonic hypoglycemia**

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

SIderoblastic anemia Isoniazid due to B6 (pyridoxine) deficiency

What are the types of hemoglobin

What does sorbitol get broken down to in healthy individuals? whats the reaction and enzymes

**Fructose** glucose--*Aldose reductase*-\> Sorbitol --*sorbitol dehydrogenase*--\> Fructose

what does galactosemia predispose to?

E. coli infections

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

These are used to induce Posttranscriptional **Silencing of genes** ## Footnote **mRNA**

What causes lactic acidosis whats the primary site of lactate clearance?

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

whats the enzyme after g6pd? turns Ribose-5-p to Fructose-6-p

Transketolase

Which vitamin gets deficient when you eat a bunch of raw eggs? what reactions does this fuck up

B7 Biotin ## Footnote **Carboxylation**

Fructose 2,6 bisphosphonate

Insuline increases F26B which inihibits glycolysis Glucagon decreases F26B which stimulates gluconeogensis

What do RAS proteins bind to?

GTP

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

Alternate RNA Splicing

Whats the deficiency in Pompe dz what findings will you see

its a Glycogen storage dz **A-glucosidase** Pompe trashes the pump (**Cardiomegaly**) **Glycogen in lysosomes**

What are the reactions that occur in the cytoplasm

HMP (pentose phosphate) Glycolysis Fatty acid Synthesis

Whats special about Primase

its a DNA dependent **RNA Polymerase** capable of making **Uracil**

Where is ketone metabolism and which tissue cant use them

Mitochondria Erythrocytes

how do erythrocytes get their energy since they dont have mitochondria

Via glycolysis but they have to make **2,3BPG**

which enzyme neutralizes free radicals in cells

**Superoxide dismutase**

Whats the **Wobble hypothesis**

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

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

**DNA polymerase I** **5-3** exonuclease

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

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**

which substance inhibits B-oxidation in a well fed state

**Malonyl-Coa**

Which ezyme helps breakdown **fructose** when you have a fructokinase deficieny

**Hexokinase**

whats the difference between Germline mosacism and somatic?

Germline mosaicims means 2 **normal parents** give birth to **affected kids** \_\_\_\_\_\_\_\_ Somatic mosaicism means an **affected parent** has **normal kids**

What is a luecine zipper?

2 alpha helical Luecine in every **7th position** its a **TRANSCRIPTION FACTOR**

What are the dz associated with VHL

RCC, hemangioblastoma, Pheo