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82 Cards in this Set

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NF- κB
transcription factor which induces cytokine prodxn in response to microbial infx
#immunology #molecules
NOD2
[Nucleotide Binding Oligomerization Domain]

intracellular microbial receptor which which triggers NF- κB transcription factor → cyokine prodxn
#immunology #molecules
Galactosyl β-1,4 - glucose
aka Lactose
#biochemistry #molecules
thiolase
enzyme which conjugates 2 acetyl-CaA → acetoacetyl-CoA

the necessary substrate for HMG CoA Synthase in the formation of cholesterol
#biochemistry #molecules #cardiovascular
Haptoglobin Levels
Haptoglobin Binds Free Hemoglobin within Blood Vessels

Complexed Hemoglobin-Haptoglobin is phagocytized by Spenic M∅

↓ serum haptoglobin levels ≈ intravascular hemolysis

normal serum haptoglobin levels rules out intravascular hemolysis (ie extravascular = direct splenic phagocytosis)
#hematology #diagnostics #molecules
Differentiating Intravascular from Extravascular Hemolysis
Haptoglobin Levels

Haptoglobin Binds Free Hemoglobin within Blood Vessels

Complexed Hemoglobin-Haptoglobin is phagocytized by Spenic M∅

↓ serum haptoglobin levels ≈ intravascular hemolysis

normal serum haptoglobin levels rules out intravascular hemolysis (ie extravascular = direct splenic phagocytosis)

NB: Extravascular includes Splenic Phagocytosis
#hematology #diagnostics #molecules
c-myc fnx
nuclear transcription factor
#molecules #pathology #neoplasia
TGFα mutation
oncogene

astrocytoma, hepatocellular carinoma
#pathology #neoplasia #molecules
What is the main mechanism for regulating iron stores?
Hepatically produced Hepcidin produced by liver binds ferroportin and causes its degradation

[Ferroportin is basolateral iron transporter on gut epithelium. No ferroportin → iron lost in gut shedding]

Hepcidin prodxn ↑ w/ high [iron] or inflammation
#nutrition #biochemistry #molecules
Hepcidin
Hepatically produced Hepcidin produced by liver binds ferroportin and causes its degradation

[Ferroportin is basolateral iron transporter on gut epithelium. No ferroportin → iron lost in gut shedding]

Hepcidin prodxn ↑ w/ high [iron] or inflammation

Main mechanism of iron regulation
#nutrition #biochemistry #molecules
Hemosiderin
aggregation of ferritin micelles

maker of iron accumulation

golden yellow brown pigment

indicative of chronic iron overload/hemolytic anemia
#pathology #molecules #diagnostics #hematology
Pigment found in chronic iron overload
Hemosiderin

aggregation of ferritin micelles

golden yellow brown pigment

indicative of chronic iron overload/hemolytic anemia
#pathology #molecules #diagnostics #hematology
3-Hydroxy-3-methylglutaryl- lyaseCoA
HMG CoA Lyase

responsible for ketogenesis from HMG CoA (which itself is produced yby degradation of leucine or synthesis via HMG coA synthase)
#biochemistry #molecules
nasal transepithelial potential difference
a sensitive test for Cystic Fibrosis (more sensitive than Cl- sweat test)

test is positive if difference is more negative

CFTR both secretes chloride and inhibits Na+ secretion

reduced CFTR fnx → ↓ Cl- secretion ↑ Na+ secretion

more negative nasoepithlial surface

NB: sweat glands are the only glands where CFTR fnx to resporb Cl-
#pathology #molecules #diagnostics #geneticdzs
How is glycogen synthase activated
Insulin → tyrosine kinase → protein phosphatase-1 →

dephosphyrlates glycogen synthase (activating)

also

dephosphorylates Fructose 1,6 bisphosphatase, inactivating and inhibiting gluconeogeneis
#biochemistry #energymetzm #molecules
protein phosphatase-1
Insulin → tyrosine kinase → protein phosphatase-1 →

dephosphyrlates glycogen synthase (activating)

also

dephosphorylates Fructose 1,6 bisphosphatase, inactivating and inhibiting gluconeogeneis
#biochemistry #energymetzm #molecules
Transporter Defect in Cysteinuria
Dibasic Cation Transporter

COAL
Cystine
Ornithine
Arginine
Lysine
#pathology #nephrology #nephrolithiasis #molecules
What is the Nitrogen Source for the Urea Cycle?

How does that molecule originally get its nitrogen?
Nitrogen Source for Urea Cycle is Aspartate (combines with Citrate to form arginosuccinate)

Oxaloacetate + NH3 = Asparatate

The NH3 for that reaction comes from Glutamate via B6

(NB: Glutamate losing NH3 becomes α-ketoglutarate)

And how does glutamate get its NH3+ you ask?

Substrate specific transaminases like AST and ALT (the two most significant):
Alanine → Pyruvate
Aspartate → Oxaloacetate
#biochemistry #energymetzm #molecules
Most common Amino Acid in Collagen
Glycine every 3rd position

Strx is always
(Gly-X-Y)x333

Proline also essential, but not as frequent
#biochemistry #molecules
Signal which gathers Myofibroblasts at wound
MMP's gather myofibroblasts at wound edges
#physiology #molecules
Tocopherol
Vitamin E
#nutrition #molecules
Vitamin E molecule name
Tocopherol
#nutrition #molecules
Ki 67
protein expressed in S phase

maker for proliferation

near 100% in cancers
#pathology #neoplasia #molecules #diagnostics
bisphosphoglycerate mutase
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping

[I guess BPG mutase fnx is upregulated by hypoxia]
#biochemistry #hemoglobin #molecules #energymetzm
1,3 BPG → 2,3 BPG
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping
#biochemistry #hemoglobin #molecules #energymetzm
2,3-bisphosphoglycerate
bisphosphoglycerate mutase
present only in RBC's & Placenta
alternative glycolytic pathway which produces no ATP

BPG mutase: 1,3 BPG → 2,3 BPG

Phosphatase: 2,3 BPG → 3 Phosphoglycerate

Bypasses one step by Phosphoglycerate kinase which would have produced ATP

2,3 BPG allosterically induces a right shift → O2 dumping
#biochemistry #hemoglobin #molecules #energymetzm
Topoisomerase I vs II
1: single stranded nicks to relieve supercoiling

2: transient doublestranded breaks

Topoisomerase II is the target of etoposide and podophyllin
etoposide esp useful for testicular cancer and small cell lung cancer
#molecules #pharmacology #neoplasia
Dihydropyridine Clalcium Channels
L type calcium channels goddamn it
#molecules #physiology #pharmacology
Naked Viral RNA can be processed by human Ribosomes
must be single stranded and positive sense
#microbiology #molecules
haptoblogin
a plasma protein which binds free hemoglobin in order to prevent its renal excretion

5d λ of haptoglobin alone
1h λ bound to heme

↓↓↓ in hemolytic anemias
#molecules #biochemistry
What biologically active molecules are derived from Tryptophan?
B6 dpt conversion to Niacin
→ NAD+ and NADP+

BH4 dpt conversion to Serotonin
→ Melatonin
#biochemistry #molecules
What biologically active molecules are derived from Glycine?
B6 dpt combination with Succinyl CoA to dALA
#biochemistry #molecules
What biologically active molecules are derived from Arginine?
Creatinine
Urea
& NO
#biochemistry #molecules
What biologically active molecules are derived from Glutamate?
Glutathione
B6 dpt conversion to GABA
#biochemistry #molecules
What is the necessary precursor molecule for DOPA synthesis?
Tyrosine

BH4 dpt conversion to DOPA
#biochemistry #molecules
What is the necessary precursor molecule for Tyrosine synthesis?
Phenylalanine

BH4 dpt conversion to Tyrsoine


Tyrosine may be converted to Thyroxine or DOPA*

[DOPA may be converted to dopamine^ or melanin]

*also req BH4
^B6 dpt
#biochemistry #molecules
What is the necessary precursor molecule for Niacin synthesis?
Trytpophan

B6 dpt conversion to Niacin
necessary for NAD+ & NADP+

Tryptophan may also undergo BH4 dpt conversion to Serotonin
#biochemistry #molecules
What is the necessary precursor molecule for Serotonin synthesis?
BH4 dpt conversion of Tryptophan

necessary for Melanonin prodxn

Tryptophan may also be converted to Niacin (w/ B6)
#biochemistry #molecules
What is the necessary precursor molecule for NO synthesis?
Arginine
#biochemistry #molecules
What is precursor molecule to Urea prodxn?
Arginine

also precursor to NO & Creatinine
#biochemistry #molecules
What is the precursor molecule to Creatinine prodxn?
Arginine

also precursor to NO & Urea
#biochemistry #molecules
What is the necessary precursor molecule for GABA synthesis?
Glutamate & B6

Glutamate also precursor to Glutathione
#biochemistry #molecules
What is the necessary precursor molecule for Glutathione synthesis?
Glutamate

Glutamate also precursor to GABA (w/ B6)
#biochemistry #molecules
FGF gene in embryogenesis
produced at apical dermal ectodermal ridge (thickened ectoderm at distal end of each developin limb). Stimulates mitosis of underlying mesoderm responsible for lengthening limbs.
#embryology #molecules
Wnt-7 gene
produced at apical ectodermal ridge (thickened ectoderm at distal end of each developin limb). Necessary for proper organization along dorsal-ventral axis
#embryology #molecules
Sonic Hedgehog Gene
produced at base of limbs in zone of polarizing activity. involved in patternining along A-P axis
#embryology #molecules
produced at base of limbs in zone of polarizing activity. involved in patternining along A-P axis
Sonic Hedgehog Gene
#embryology #molecules
produced at apical ectodermal ridge-- necessary for proper organization along dorsal-ventral axis
Wnt-7 gene

ectodermal ridge: (thickened ectoderm at distal end of each developin limb
#embryology #molecules
produced at apical dermal ectodermal ridge-- stimulates mitosis of underlying mesoderm responsible for lengthening limbs.
FGF gene

ectodermal ridge: (thickened ectoderm at distal end of each developin limb
#embryology #molecules
PABA
para-aminobenzoic acid

folic precursor in prokaryiotes

sulfas are PABA analogues

humans lack abilityt o convert PABA to folic acid, thus require folate
#microbiology
#pharmacology #antibiotics
#biochemistry #molecules
Neurophysin
carrier proteins for oxytocin and vasopressin
#physiology #biochemistry #endocrinology #molecules
GP Ib-IX
platelet receptor for vWF
#biochemistry #hematology #molecules
Calcitriol
aka active form of vitamin D

1,25-dihydrocholecalciferol
#physiology #molecules
Eythrocyte transketolase
↑ activity after thiamin infusion diagonistic of thiamin deficiency
#vitamins #molecules
Dz: Fabry's Dz
(Lysosomal Storage Dz)
Presentation: Peripheral Acorneuropathy, angiokeratomas, Cardiovascular/Renal Dz
Deficiency: alpha galactosidase A
Accumulation: Ceramide tihexose
Inheritance: XR --NB the only X linked lysosomal storage dz
#pathology #molecules #geneticdzs
Dz: Gaucher's Dz
(Lysosomal Storage Dz)
Presentation: HSM, Asepptic necorsis of femur; Gaucher's cells: macorphages that look like crumpled ts paper
Deficiency: Glucocerebrosidase
Accumulation: Glucocerebroside
Inheritance: AR (all but Fabry's & Hunters AR)
NB: most common Lysosomal stroage Dz
#pathology #molecules #geneticdzs
Dz: Neimann-Pick
(Lysosomal Storage Dz)
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM (defining feature vs. Tay-Sach's)
Deficiency: sphingomyelinase
Accumulation: sphingomyelin
Inheritance: AR (All but Fabry's & Hunters AR)
Mn: Neiman picks his nose with hi-sphinger
#pathology #molecules #geneticdzs
Dz: Tay-Sach's
(Lysosomal Storage Dz)
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin, no HSM (defining feature vs. Niemann Pick)
Deficiency: Hexosaminidase A
Accumulation: GM2 ganglioside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Dz: Krabbe's Dz
(Lysosomal Storage Dz)
Presentation: Peripehral neuropathy, dvlptl delay, optic atrophy, globoid cells
Deficiency: beta galactocebrosidase
Accumulation: galactoserebroside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Dz: Hurler's
(Lysosomal Storage Dz)
Presentation: Dvlpt delay, gargolylism, ariway obstrx, corenal clouding, HSM
Deficiency: alpha L iduronidase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Dz: Hunter's
(Lysosomal Storage Dz)
Presentation: aggressive behavior mild dvlpt delay, HSM
Deficiency: Iduronidate sulfatase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Ashkenazi Jews
Dz: Gaucher's Dz
(Lysosomal Storage Dz)
Presentation: HSM, Asepptic necorsis of femur; Gaucher's cells: macorphages that look like crumpled ts paper
Deficiency: Glucocerebrosidase
Accumulation: Glucocerebroside
Inheritance: AR (all but Fabry's & Hunters AR)
NB: most common Lysosomal stroage Dz


Dz: Neimann-Pick
(Lysosomal Storage Dz)
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM (defining feature vs. Tay-Sach's)
Deficiency: sphingomyelinase
Accumulation: sphingomyelin
Inheritance: AR (All but Fabry's & Hunters AR)
Mn: Neiman picks his nose with hi-sphinger

Dz: Tay-Sach's
(Lysosomal Storage Dz)
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin, no HSM (defining feature vs. Niemann Pick)
Deficiency: Hexosaminidase A
Accumulation: GM2 ganglioside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Deficiency: alpha galactosidase A
Dz: Fabry's Dz
(Lysosomal Storage Dz)
Presentation: Peripheral Acorneuropathy, angiokeratomas, Cardiovascular/Renal Dz
Deficiency: alpha galactosidase A
Accumulation: Ceramide trihexose
Inheritance: XR --NB the only X linked lysosomal storage dz
#pathology #molecules
Deficiency: Glucocerebrosidase
Dz: Gaucher's Dz
(Lysosomal Storage Dz)
Presentation: HSM, Asepptic necorsis of femur; Gaucher's cells: macorphages that look like crumpled ts paper
Deficiency: Glucocerebrosidase
Accumulation: Glucocerebroside
Inheritance: AR (all but Fabry's & Hunters AR)
NB: most common Lysosomal stroage Dz
#pathology #molecules
Deficiency: sphingomyelinase
Dz: Neimann-Pick
(Lysosomal Storage Dz)
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM (defining feature vs. Tay-Sach's)
Deficiency: sphingomyelinase
Accumulation: sphingomyelin
Inheritance: AR (All but Fabry's & Hunters AR)
Mn: Neiman picks his nose with hi-sphinger
#pathology #molecules
Deficiency: Hexosaminidase A
Dz: Tay-Sach's
(Lysosomal Storage Dz)
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin, no HSM (defining feature vs. Niemann Pick)
Deficiency: Hexosaminidase A
Accumulation: GM2 ganglioside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Deficiency: beta galactocebrosidase
Dz: Krabbe's Dz
(Lysosomal Storage Dz)
Presentation: Peripehral neuropathy, dvlptl delay, optic atrophy, globoid cells
Deficiency: beta galactocebrosidase
Accumulation: galactoserebroside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Deficiency: alpha L iduronidase
Dz: Hurler's
(Lysosomal Storage Dz)
Presentation: Dvlpt delay, gargolylism, ariway obstrx, corenal clouding, HSM
Deficiency: alpha L iduronidase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Deficiency: Iduronidate sulfatase
Dz: Hunter's
(Lysosomal Storage Dz)
Presentation: aggressive behavior mild dvlpt delay, HSM
Deficiency: Iduronidate sulfatase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Accumulation: Ceramide trihexose
Dz: Fabry's Dz
(Lysosomal Storage Dz)
Presentation: Peripheral Acorneuropathy, angiokeratomas, Cardiovascular/Renal Dz
Deficiency: alpha galactosidase A
Accumulation: Ceramide trihexose
Inheritance: XR --NB the only X linked lysosomal storage dz
#pathology #molecules
Accumulation: Glucocerebroside
Dz: Gaucher's Dz
(Lysosomal Storage Dz)
Presentation: HSM, Asepptic necorsis of femur; Gaucher's cells: macorphages that look like crumpled ts paper
Deficiency: Glucocerebrosidase
Accumulation: Glucocerebroside
Inheritance: AR (all but Fabry's & Hunters AR)
NB: most common Lysosomal stroage Dz
#pathology #molecules
Accumulation: sphingomyelin
Dz: Neimann-Pick
(Lysosomal Storage Dz)
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM (defining feature vs. Tay-Sach's)
Deficiency: sphingomyelinase
Accumulation: sphingomyelin
Inheritance: AR (All but Fabry's & Hunters AR)
Mn: Neiman picks his nose with hi-sphinger
#pathology #molecules
Accumulation: GM2 ganglioside
Dz: Tay-Sach's
(Lysosomal Storage Dz)
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin, no HSM (defining feature vs. Niemann Pick)
Deficiency: Hexosaminidase A
Accumulation: GM2 ganglioside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Accumulation: galactoserebroside
Dz: Krabbe's Dz
(Lysosomal Storage Dz)
Presentation: Peripehral neuropathy, dvlptl delay, optic atrophy, globoid cells
Deficiency: beta galactocebrosidase
Accumulation: galactoserebroside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Accumulation: heparan sulfate, dermatan sulfate
Dz: Hurler's
(Lysosomal Storage Dz)
Presentation: Dvlpt delay, gargolylism, ariway obstrx, corenal clouding, HSM
Deficiency: alpha L iduronidase
Accumulation: heparan sulfate, dermatan sulfate (same as Hunter's)
Inheritance: AR (All but Fabry's & Hunters AR)

Dz: Hunter's
(Lysosomal Storage Dz)
Presentation: aggressive behavior mild dvlpt delay, HSM
Deficiency: Iduronidate sulfatase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Presentation: Peripheral Acorneuropathy, angiokeratomas, Cardiovascular/Renal Dz
Dz: Fabry's Dz
(Lysosomal Storage Dz)
Presentation: Peripheral Acorneuropathy, angiokeratomas, Cardiovascular/Renal Dz
Deficiency: alpha galactosidase A
Accumulation: Ceramide trihexose
Inheritance: XR --NB the only X linked lysosomal storage dz
#pathology #molecules
Presentation: HSM, Aseptic necorsis of femur; macorphages that look like crumpled ts paper
Dz: Gaucher's Dz
(Lysosomal Storage Dz)
Presentation: HSM, Asepptic necorsis of femur; Gaucher's cells: macorphages that look like crumpled ts paper
Deficiency: Glucocerebrosidase
Accumulation: Glucocerebroside
Inheritance: AR (all but Fabry's & Hunters AR)
NB: most common Lysosomal stroage Dz
#pathology #molecules
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM
Dz: Neimann-Pick
(Lysosomal Storage Dz)
Presentation: progressive neuodegeneration, cherry red spot on macula, foam cells, HSM (defining feature vs. Tay-Sach's)
Deficiency: sphingomyelinase
Accumulation: sphingomyelin
Inheritance: AR (All but Fabry's & Hunters AR)
Mn: Neiman picks his nose with hi-sphinger
#pathology #molecules
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin
Dz: Tay-Sach's
(Lysosomal Storage Dz)
Presentation: Prgoressive neurodegeneration, cherry red spot on macula, lysosomes with onion skin, no HSM (defining feature vs. Niemann Pick)
Deficiency: Hexosaminidase A
Accumulation: GM2 ganglioside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Presentation: Peripehral neuropathy, dvlptl delay, optic atrophy, globoid cells
Dz: Krabbe's Dz
(Lysosomal Storage Dz)
Presentation: Peripehral neuropathy, dvlptl delay, optic atrophy, globoid cells
Deficiency: beta galactocebrosidase
Accumulation: galactoserebroside
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Presentation: Dvlpt delay, gargolylism, ariway obstrx, corenal clouding, HSM
Dz: Hurler's
(Lysosomal Storage Dz)
Presentation: Dvlpt delay, gargolylism, ariway obstrx, corenal clouding, HSM
Deficiency: alpha L iduronidase
Accumulation: heparan sulfate, dermatan sulfate (same as Hunter's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules
Presentation: aggressive behavior mild dvlpt delay, HSM
Dz: Hunter's
(Lysosomal Storage Dz)
Presentation: aggressive behavior mild dvlpt delay, HSM
Deficiency: Iduronidate sulfatase
Accumulation: heparan sulfate, dermatan sulfate (same as Hurler's)
Inheritance: AR (All but Fabry's & Hunters AR)
#pathology #molecules