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HUCM Proteins 3
Description
44
Medical
Graduate
08/13/2011

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Term
? is a disease characterized by the buildup of misfolded proteins called prion proteins in the brain
Definition
CJD ( Creutzfeld-Jacob Disease)
Term
Quaternary structure is stabilized by ?-? forces only.
Definition
non-covalent
Term
Hemoglobin, Aspartate Transcarbamylase, lactate dehydrogenase, Phosphorylase b kinase, and Immunoglobulin G are examples of some ? ?
Definition
oligomeric proteins
Term
Developmental and tissue specificity is common in ?
Definition
enzymes
Term
Lactate Dehydrogenase (LDH) can exist as a ? or a heterotetramer of different combinations of two types of subunits, M and H
Definition
homotetramer
Term
What is a tetramer of four identical subunits, with four catalytic sites as well as regulatory sites, and it is allosterically INHIBITED by ATP, citrate; ACTIVATED by AMP, Fructose-2,6-BP
Definition
Phosphofructokinase 1
Term
What enzyme is the key control point for glycolysis
Definition
Phosphofructokinase-1
Term
What catalyzes first committed step in pyrimidine nucleotide biosynthesis, formation of N-carbamoylaspartate and Pi from carbamoyl phosphate and aspartate
Definition
Aspartate Transcarbamoylase (ATCase)
Term
What enzyme has 2 catalytic trimer (C3) and 3 regulatory dimers (r2) come together to form C6r6 complex and is inhibited (feedback) by CTP; activated by ATP and is also an example of ALLOSTERIC enzyme
Definition
Aspartate Transcarbamoylase (ATCase)
Term
One advantage of not being single for a protein is regulation through ? ? (Hb, PFK-1, ATCase)
Definition
Cooperative Effects
Term
One advantage of not being single for a protein is channeling of substrates between different ? ? - multienzyme complexes
Definition
functional sites
Term
Do proteins spontaneously adopt a folded conformation after they are synthesized by the ribosome of the cell?
Definition
Often times yes sometimes they need added help
Term
What bind to unfolded and partially folded proteins and prevent improper association? Ex HSP-70 and chaperonins
Definition
Chaperones
Term
Folded states are in equilibrium with unfolded states, though equilibrium is skewed toward the ? ?.
Definition
folded state
Term
Chemical denaturants include ?, Guanidine HCl, and Sodium dodecyl sulfate. Other denaturing agents are heat, low temp, and pH extremes
Definition
Urea
Term
S-S bonds can be broken with beta-mercaptoethanol or ?
Definition
dithiothreitol (DTT)
Term
The irreversible in vivo accumulation of unfolded or misfolded proteins is the under-lying cause of a class of degenerative diseases called ?
Definition
amyloidoses
Term
Examples of amyloidoses are ? ?, mad cow disease (Bovine Spongiform Encephalopathy), Creutzfeld-Jacob disease, other neurodegenerative disorders
Definition
Alzheimer's disease
Term
The belief on prions was heretical because it goes against accepted dogma which says that ? ? must be involved and these proteinaceous infectious particles lack that.
Definition
nucleic acid
Term
Gerstmann-Straussler-Scheinker disease, familial CJD, Fatal familial insomnia are all ? ? in Prp gene
Definition
Germline mutations
Term
What is the mechanism for kuru?
Definition
Ritualistic cannibalism
Term
The mechanism for variant CJD is?
Definition
Bovine prions
Term
The mechanism for sporadic CJD is ?
Definition
Somatic mutation or spontaneous conversion of PrPc to PrPsc
Term
Latrogenic (hospital caused) CJD is due to ?-? HGH, grafts, etc
Definition
Prion-contaminated
Term
PrPsc is the ? form
Definition
infectious
Term
PrPc to PrPsc involves conversion of two ? ? to ??
Definition
alpha helices to beta strands
Term
Accumulation of ? always associated with pathology of prion diseases
Definition
PrPsc
Term
Knock out of PrP gene eliminates substrate for PrPsc formation, and prevents prion disease - no ?
Definition
abnormalities
Term
PrP gene mutations genetically linked to ? prion disease
Definition
inherited
Term
Glycosidic addition catalyzed by specific ?
Definition
glycosyltransferases
Term
3 roles of glycosylation in glycoproteins include:
1. proper folding of transmembrane proteins.
2.structural stability
3.?
Definition
Recognition for cell adhesion
Term
Blood group A has extra ?
Definition
GalNAc
Term
Blood group B has extra ?
Definition
Gal
Term
Protein ? promotes resistance to degradation in cell and helps control gene expression in eukaryotes
Definition
Acetylation
Term
Three common types of attachment for lipidation are: acylation, prenylation, and ?
Definition
glycosylphosphatidylinositl
Term
What two things modulate phosphorylation?
Definition
Kinases and phosphatases
Term
? is common in collagen and elastin; catalyzed by hydroxylases
Definition
Hydroxylation
Term
Carboxylation of glutamic acid is often seen in ? ? ?
Definition
blood clotting factors
Term
Carboxylation requires ? ? as enzyme cofactor
Definition
Vitamin K
Term
N-linked or O-linked methylation of AA with distal amino acids exhibited by ? and ?
Definition
Arg and Lys
Term
N-linked or O-linked methylation of carboxyl groups exhibited by ? and ?
Definition
Asp and Glu
Term
? use energy released from ATP hydrolysis to move specific ions against an electro chemical gradient. Also an example?
Definition
Pumps
Ex. Na+/K+ pump
Term
? permit the movement of ions down their electrochemical gradient (passive transport) Ex?
Definition
Channels
Nongated ion channels (Na+, Ca++, Cl-)
Term
? facilitate movement of specific small molecules or ions. Ex?
Definition
Transporters
GLUT 1 - 12 (ex. of uniporter which transport a single molecule down its conc gradient)
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