STEP 1: Biochem

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NADPH Production: HMP Shunt
(Non-oxidative pathway)
Ribulose-5-Phosphate Think ___, and _____.: 1. Need for NADPH
2. Cholesterol biosynthesis in liver
Fasting after 4 hours: blood glucose is maintained by _________ think________ --> _______: Gluconeogenesis
Oxaloacetate to phenylpyruvate
Ketogenesis: Think these 3 things:: 1. Phosphorylation/dephosphorylation of acyl CoA
2. Activated by glucagon
3. Deactivated by Insulin
In protein catabolism, amino acids lose their ____ to compounds like ____ in a _____ reaction.: Nitrogen, glutamate, transamination

This nitrogen is then used to form ammonia and is excreted in urine after conversion to urea
Photosensitive Porphyrias occur in later exzyme defieciencies These will include: (4): 1. URO decarboxylase,
2. Copro Oxidase,
3. Ferrochelotase,
4. Proto-oxidase
ascorbic acid =: Vitamin C
Vit B2 = Lack of causes:: Riboflavin
Cheilosis, stomatitis, glossitis, dermatitis

Think pt with small cuts in corner of mouth and swollen tongue
Folic Acid Deficiency =: Megaloblastic anemia and neural tube defects in fetus
Need Folate (= vit B____) For ___, ___ and _____.: Nucleotide biosynthesis, remethylation of homocystein, and DNA repair
Zinc deficiency: Look for ____, ____, and ____: acrodermatitis, growth retardation, infertility
Tyrosine is present in the body via ___ or ____ and is the precursor for _______.: Ingestion or derived from Penylalanine.
Catecholamines:
DOPA, Dopamine, Norepinephrine, Epinephrine
Unlike ____ and ____, _____ is derived almost soley in the adrenals.: Dopamine and norepinephrine,
Epinephrine is derived mainly in the adrenals
Converts Nore-epi --> Epi Is up regulated by _____: PNMT
Cortisol
Thyroid hormones act via _____ receptors: nuclear

This means that they lead to direct synthesis of new products
JAK STAT inducers: CHIP:

Cytokines, growth Hormone, IL-2, Prolactin
Thiamine is also needed for _____ which is pertinent to alcoholics: Succinyl Co-A formation from alpha-ketoglutarate
Allosterically activates gluconeogenesis: Acetyl CoA
increases activity of pyruvate carboxylase: Acetyl CoA
Sucrose = Lactise = Maltose =: S = Fructose + Glucose
L= Galactose + Glucose
M= Glucose + Glucose
Fructose intolerance = ______ deficiency: Aldolase B

Remember that Fructose is also in Sucrose - so a fructose in tolerance = a sucrose intolerance as well
Classic galactosemia = ________ impairment: glactose-1 Phosphate metabolism
Baby has FTT after breastfeeding begins. What enzyme is most likely defiecient?: Galactose-1 Phosphate Uridyl Transferase
Fructose in Urine = Is an Asymptomatic disorder due to the formation of ______ from fructose by _____: Fructose 6 P via Hexokinase

(Note Aldolase B deficiency leads to buid-up of Fructose 1 P)
CO2 carrier Also called Vitamin _____.: Biotin, B7
Biotin is needed for these 2 conversions:: 1. Pyruvate to Oxaloacetate
2. Amino Acids (Valine) to Succinyl Co A
1. Converts pyruvate in the presence of oxygen 2. pyruvate --> _______: 1. Pyruvate dehydrogenase
2. Acetyl Co-A
Amino acids that are purely ketogenic (2) ie they have no glucogenic components This means that they will not lead to the formation of ____.: Leucine and Lysine

Lactic acid
Amino acids that are both ketogenic and glucogenic: PIT: "both go in the PIT"

Phanylalanine, Isoleucine, Tryptophan
Infant with FTT and hepatomegaly following a diet change most likely has ____ which is caused by a defieciency in ________.: Essential Fructose intolerance

Aldolase B
Steroid receptors are on the cell _____, are ___(protein form)____ , and are bound to ______ such as ____ and ____.: Nucleus, Zinc Fingers, Heat shock proteins, hsp90 and hsp56
cAMP receptors are bound to a ______ that activates _____ which then activates more cAMP and thus ____.: G Protein, adenylyl cyclase, Protein Kinase A
The ultimate Goal of the IP3 pathway is to increase ________. THe pathway is: ___ --> ____ --> ____ --> ____-->____: Cytosolic Ca++

Receptor --> phospholipase C
-->IP3+DAG -->increased Ca++ --> Prot. Kinase C
Ras is part of the ______ system: MAP Kinase
After the steroid receptor complex forms, the _____ are lost and the complex _______.: Heat shock proteins (hsp 56 and 90), the complex then enters the nucleus to initiate transcription
NAD+ is rejuvenated for the anaerobic process when ___ is converted to ____: Pyruvate --> Lactate
Increased concentrations of _____ inhibit the process of glycolysis: Citrate
These cells are able to convert Sorbitol to _____ with the enzyme ____: Seminal Vessicle Cells

Fructose, sorbitol dehydrogenase

Cells that lack Sorb Deh, such as liver, kidney, retinal, lens, schwann.... are thus at greater risk in cases of high glucose - ie diabetes.
Pellagra is caused by _____ deficiency: Niacin
When not derived from food, Niacin can be made endogenously from ____.: Tryptophan
Arginine is neccessary for the creation of ____, ___ and ____.: Nitric oxide, creatine, urea
The precursor for vitamin A is ____.: Carotene
Pellagra is characterized by ___, ___ and ___, and can be found in populations that subsist solely on ____.: Dermatitis, diarrhea, and dementia.

Corn
Pyrimidine is formed from ____.: Orotic acid
GTP is produced in the TCA cycle in this step, and it is used to phosphorylate phosphoenolpyruvate in gluconeogenesis.: Succinyl Co-a --> Succinate
Valine, alanine, isoleucine + Alpha-helical arrangement =: Membrane spanning portion of membrane protein
Membrane spanning proteins are usually messengers for these types of hormones:: Glycoprotein hormones such as TSH, FSH, and LH
Creatine increases ___ in most cells of the body, is mostly stored in ______ and is first limited by the enzyme ______ in the kidneys and pancrease, and then _______ in the liver and pancrease: ATP, skeletal muscle, GATM, GAMT
Hydrophobic Amino Acids (as in the kind that could be in the membrane spanning portion of a cell membrane): PAM VI hates water

Phenylalanine, Alanine, Methionine, Valine, Isoleucine
Beta cells release insulin when the ______ ratio increases, shutting down the _____ which opens _______ .: ATP:ADP ratio
Potassium channel (KATP) shuts down
Opens Ca++ volatage gated channels which release insulin
Glucose sensor in Beta cells. Can be deficient in Gestational Diabetes.: Glucokinase
Glucise 6 Phosphatase is only present in the ____: liver
Infant with growth retardation, hepatomegaly, and hypoglycemia lacks _____: debranching enzyme
Hemolytic anemia can occur with ____ deficiency: pyruvate kinase
Muscle cramping and exercise intolerance can be caused by a lack in ______: Glycogen phosphorylase
______ deficiency causes a shunt toward _______ production from galactose via _______ causing bilateral cataracts.: Galactose Kinse deficiency causes increased Galactitol production via Aldose reductase.

Increased Galctitol causes cataracts
Vitamin _____ is useful in treating Measles. Think child from ______.: A, deficiency is common in Asia, Africa, and S America
Tryptophan is the precursor for ____, ____ and _____.: Nicotinic Acid, serotonin, melatonin.
Tryptophan deficiency is called ________. Children with the intestinal absorptive issues of this disease have _______ symptoms because tryptophan is a precursor for ______.: Hartnup disease
Pellagra-like
Niacin
_____ bonds in ______ cause recoil abilities in ______.: Lysine, elastin, Alveolar
HbC Disease is caused by a ______ mutation that switches the normal _____ to a _____.: missense, glutamine (normal) is switched to Lysine
IN HbS, ______ is switched to ____.: Glutamic acid is swtched to valine
Movement of unconjugated bilirubin into the liver is ______ and movement of conjugated bilirubin to be excreted is ____.: passive, direct bilirubin excretion is active.
Unconjugated bilirubin is _____ and must be bound to ____.: not water soluble, must be bound to albumin
Fetal blood has 50% _______ and takes _____ to switch to HbA.: HbF, 6 months
______, formed from ______, is a methyl donor.: SAM, methionine
The START codon for all ribosomal translations is ____, which codes for ____. Thus, it is present at all the ______.: AUG, methionine, N-terminal position for all proteins
Homocystien is converted to ____.: Methionine
Homocysteinuria occurs in ____ deficiency. Pts cannot make ____.: Vitamin b12 / cobalamin
Methionine
Periodic Hemolytic anemia can be cause by _____ or ______ deficiency.: G6PD, Glutathione reductase
Chronic Hemolytic anemia will most often (95%) be caused by a ____ deficiency.: pyruvate Kinase
Pyruvate dehydrogenase requires ___, ____, ___, ___and ____ as cofactors.: Thiamine, lipoic acid, Coa, FAD, NAD+
Without lipoic acid there will be ____ because ____ cannot be converted to ____.: lactic acidosis, pyruvate cannot be converted to acetyl Co-A

The pyruvate is shunted towards lactate formation
The Hemoglobin Curve shifts RIGHT when these three things ______.: INCREASE in
Temperature,
2,3 DPG
H+/ acid
THe hemoglobin curve shifts LEFT when these three things _____.: DECREASE
Temperature
2,3 DPG
H+ (more basic)
A decreased P50 causes ______ because ____ oxygen is released to tissues.: REFLEX polycythemia because LESS oxygen is released to tissues
2,3 BPG is in _____ and increases when blood oxygen levels are ____ in order to ____ oxygen movement INTO tissues.: RBCs, LOW, INCREASE

When blood oxygen is low, 2,3 BPG ensures that the oxygen available will be moved INTO the tissues.
Spoon nails (koilinychia) and difficulty swallowing (dysphagia) = ____: Iron deficiency anemia
heme synthesis in the liver is used for _____ as opposed to heme produced in the bone marroe, which is used for ___: liver: cytochrome P450 system
marrow: hemoglobin synthesis
decreased heme will ____ hepatic ALA synthase activity. This can cause ___, ___, and ___.: increaes
abdominal pain, neurological issues, dark urine with standing
Acute intermittent porphyria is an _______ disease cause by _____ deficiency.: autosomal dominant, porphobilinogen deaminase.

The low heme levels increase ALA synthase activity. Thus, is can be treated with heme components.
Acute porphyria attacks can be brought on my ____ (3)_____.: Alcohol, barbituates, hypoxia
(also a low calorie diet)
RBCs use the HMP shunt to _______.: Maintain high levels of NADPH to keep glutathione reduced
G6PD is a common _________ disease (think African Americans) that can cause acute anemia. A common symptom is ____. ____ is not formed.: X-linked
Dark urine
6 phosphogluconate
G6PD deficiency leads to RBC destruction because of ____.: oxidative stress
UTI = ____ as treatment which can cause ______ leading to an anemic episode in patients with ____.: trimethoprim-sulfamethoxazol
oxidative hemolysis
G6PD deficiency
The formation of ______ stabilizes the hemoglobin molecule and allows for release of oxygen at the tissue level. These form when ____ is high, which means that _____ are available. (pH is ___): salt bridges, CO2 is high, protons (lowered pH)

Histidine amino groups on the hemoglobin molecule can be protonated, forming salt bridges that stabilize the de-oxy hemoglobin molecule
In the lungs, hemoglobin binds ___ and loses ___.: Lungs:
Binds oxygen, loses protons
O2 partial pressure in blood is ____.: the amount of oxygen dissolved in the plasma.

It is NOT related to hemoglobin O2 saturation
Ferrous to _____ iron on the heme molecule leads to the formation of ____. This _____bind oxygen.: Ferric (3+)
Forms Methemeglobin which CAN NOT bind oxygen
UV damage causes ____ formation which is corrected by ___.: pyrimidine dimer, endonuclease repair in which the damaged region is nicked and replaced.
CO poisoning is dangerous because it has a _________.: 200xs greater affinity for hemoglobin than oxygen, and once bound, is irreversible.
a-ketoglutarate to Succinyl Co-A requires ___.: Thiamine

In an alcoholic vignette for biochem, think a-ketog to Succ Co-a
Pantothenic acid = _____ Think ___ --> ____ in the TCA cycle: Co-enzyme A

Oxaloacetate to Citrate
HbF does not bind ____. HbF will continue to be produced if there is a mutation where ___ is replaced with ___.: 2,3 DPG (in order to get blood from the placenta)

histidine, serine
1. Rate limiting enzyme of the HMP shunt 2. Deficiency causes _____: 1. G6PD
2. Hemolytic Anemia

Can be exacerbated by sulfonamides, anti-malarials, FAVA BEANS
____ and _____ carry out the non-oxidative functions of the HMP shunt. They allow for conversion of ___ to ____.: Transaldolase and Transkelotase

Fructose-6-phosphate to Ribose.
the HbS mutation allows for _________ in _____ or ____ conditions: hydrophobic interaction between Hgb molecules in low oxygen or acidic conditions
Glutamate carboxylation is carried out by Vitamin ___.: K
Transkelotase requires _____, is part of the _______ which is carried out in the _____.: Thiamine, Pentose Phosphate Pathway, CYTOSOL
If the individual sub units of hemoglobin were dissociated, individually they would have a much _____ oxygen affinity and ____ P50. Their curve would be sinilar to that of ____.: HIgher O2 affinity = LOWER P50

Shifts FAR left like MYOGLOBIN
3 STOP codons: UGA
UAG
UAA
Infant with Cardiomegaly + polysaccharide deposits in muscle = ____ deficiency: a-glucosidase
RBCs use ____ to deal with the high CO2 concentration of venous blood. The action of this enzyme causes higher levels of __ in the RBCs: Carbonic anhydrase

RBC Chloride levels are high in venous blood
HbF is made up of ____ and ___. Therefore, in ___ thalassemia, the infant will be normal for the first 6 months.: alpha and gamma HgB

Beta
Maturing erythrocytes cannot produce heme when they lose their ___.: mitochondria

The 1st and last 3 steps of heme synthesis occur in the mitochondria
The greenish color in bruises is caused by the formation of ____ via ____.: Bilverden, heme oxygenase
Nitrites (ie amyl nitrite) work to resolve deathly affects of cyanide by causing the formation of _____ which binds cyanide more readily, thus keeping away from _____.: Methemeglobin (ferric hemoglobin)
Mitochondrial cytochrome complex
Radiation treatment causes _____ and the formation of ____.: Double strand DNA breakage
Free radicals
Collagen structure is ____.: Triple Helix: Gly-X-Y
Nitrogen in urea is derived from ____ and ___.: NH3 and Aspartate

"A Spartan in the NHL needs to pee."

Carbamoyl Synthetase Phosphate 1 is the rate limiting enzyme in urea synthesis.
______ is elevated in Lead poisoning and is neccessary for the production of _____.: Pyridoxal Phosphate
d-Aminolevulinic Acid
___________ participates in amino acid transfer. Think oxaloacetate + Glutamate --> Aspartate: Pyridoxal Phosphate
(Pyridoxine)
Cytosine Deamination = ______: Uracil Formation
____ activates carbamoyl phosphate synthetase 1. It is Formed from ______ and ____.: N-Acetyl Glutamate

Acetyl CoA, Glutamate
____ becomes arthritis in adults. (Think darkening of ears). It is caused by a lack of ____.: Alkoptonuria
Homogentisic acid oxidase

The accumulated homogentisic acid cases brown spots throughout the body.
Bacterial Polymerase Function: 1: _____ 3: _____: 1. Removes RNA primer, repars knick of removing primer
3. Removes mismatched nucleotides
Joins Laggin (Okazaki) fragments: DNA Ligase
The Lac operon is turned OFF when ______ or when _____.: Glucose is present, cAMP is low
Enteropeptidase is neccessary for the formation of ______.: Trypsin

It is formed from trypsinogen in the duodenal wall.
____ is converted to urea via ___.: Arginine, Arginase

Deficiency causes a spasticity and choreoid movement similar to that seen in Cerebral Palsey
Increased Serum methionine could mean ____ deficiency which leads to absence of ____.: Cystethionine Synthetase
Cysteine
_____ (BH4) is a cofactor for the production of ______(4).: Tetrahydrobiopterin

Serotonin
Nitric Oxide
Tyrosine
Dopa
These 3 enzymes require 5 cofactors. NAD, FAD, Thiamine, Lipoate, Coenzyme A: a-ketoacid dehydrogenase (Maple Syrup)
Pyruvate dehydrogenase
a-ketoglutarate dehydrogenase
Ehlors Danlos occurs with a defect in ____. Think of these 2 symptoms.: Collagen
Hypermobile Joints, Elastic skin
Bacterial mRNA can be ____.: Polycistronic

1mRNA codes for several proteins
Thiamine deficiency can be determined by measuring erythrocyte ____ levels.: Transkelotase
Lactose may also be written as ____: Galactosyl b 1,4-Glucose
"mousy" odor and European descent = ____: PKU

They need TYROSINE
Blck Urine = ______ = ____ deficiency: Alkaptonuria, homogentisate oxidase
Histone _____ is located OUTSIDE of the nucleosome.: H1
Vitamin C is required to avoid ____ and is used in collagen synthesis within the ___.: Scurvy
RER
Amino group + a-ketoglutarate = _____: Glutamate

Glutamate carries the amino group that results in amino acid catabolism
Methlmalonyl CoA is an isomer of ____ which can enter the TCA cycle. Methymalonic aciduria is caused by ___ deficiency.: Succinyl CoA

Isomerase
Ornithine transport is part of _____.: Urea formation / ammonia conversion following amino acid degradation
Very long chain fatty acid oxidation occurs in ____. If these structures are absent, there will often be ____ issues due to problem with _____.: Peroxisomes

Neurological issues develop due to an inability to properly myelinate neurons

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Number of cards 136

Created By evrigsby