Biochem Day 2

Chapter 10 Contd

Retinoic Acid Receptor
- Acute promyelocytic leukemia -> t(15,17)
- Treat m3 with all trans retinoic acid therapy
- Induces differentioation of promyelocytes to myelocytes to metamyelocytes
to bands to pmn
- Person given cumerain drug or warfarin -> carboxylation process is
disturbed

Chapter 17

- Cholera toxin -> MOAaua
- Primary trigger for acid release from parietal cells: histamine, ACH, Gastrin
- Chief cells secrete pepsinogen which is autoactiveated to pepsin (20% of
total digestion)
- Trypsinogen and chymotrypsinogen
o Enteropeptidase actives to trypsin and chymotrypsin
- 3 transporters for amino acids: acidic, basic, and neutral
- peptide transporter : pep t1

Hartnup Disease
- defective neutral amino aid transporter -> intestine and kidney
- due to def intestinal uptake of tryptophan which is need to make niacin
- symptoms: failure to thrive, photo sensitivity, pellagra like rash
- 3 d of B3 -> dermatitis, diarrhea, dementia
- Treat with high protein diet -> peptides from diet contain tryptophan

Energy from amino acids
1. remove NH2 group - > releaseing Alpha ketoacid: aminotransferase
(requires vitamin b6)
2. Can oxidize carbon skeleton -> used for glucose synthesis

Nitrogen homeostasis
- toxic in brain
- moves nitrogen to nontoxic inactive forms
o Alanine
o Glutamine
o Urea
3 enymes to convert ammonia into organic nitrogen as AA:
- Glutamate dehydrogenase (GDH)
- Glutamine synthetase
- Glutaminase
In cases of Liver injury the intestine can contribute to up to 50% of sugar for the
body

In cases of metabolic acidosis -> increase glutamine into kidney -> uses more
hydrogen -> excreted -> leading to less hydrogen -> increase in PH

Cystinuria:
- defective cysteine reabsorption in kidneys
- excess cysteine in urine -> renal calculi (most common cause is dehydration)
- Disulfide dimer by oxidation of cysteine
- Treat w/ hydration and alkalizine urine w. citrate or acetazolamide

N-acetylglutamate synthase (allosteric activated by arginine) -> creates n acetyl
glutamates, which activates carbamoyl phosphate synthase

Tyrosine product reactions:
- to melanin by tyrosinase and leads to albinism
Glutamate glutamine cycle w/ edema of brain fill in

Methionine synthase need vit b12 along with methymalonyl coa mutase
Oriounoyl coa carboxylase (biotin) -> ketotic hyper glycinemia, episodic severe
ketoacidosis side effects

VOMIT pathway

Homocystinuria
- b6 def and CBS
o methionine increase -> methioninemia
- Folate def
o Decrease in methionine due to the fact that u cant create methionine
o Can lead to megaloblastic
- B12 def
o Decrease methionine
o Effects methylmalonyl coa -> methylmalonic academia
o Can lead to megaloblastic anemia

Look up tetrahydrobiopeterin

1 carbon pool serine and glycine
folate def -> megaloblastic anemia

Most common cause of folate -> alcohol abuse

85% of heme synthesis in erythrocytes and the rest in the liver
- porphyrias:
o hepatic
o erythropoietic
Microcytic anemia
- if rings sideroblasts are present it is not iron def anemia
Iron metabolism:
- Rda 15mg/day
- Only 1mg/day is absorbed

WHAT IS HEPCIDIN figure it out
Ceruloplasmin

Chapter 12

SGLT 1 -> transports glucose into enterocytes