Lecture 3 Flashcards
There are 2 possible models explaining the organization of the golgi and the transport of proteins from one cisternae to the next.
- _____ transport model
- _____ maturation model
- VESICULAR transport model
2. CISTERNAL maturation model
____ II coated vesicles that bud off from the RER shed their coat and fuse together to form vesicular tubular clusters that move along MTs toward the golgi via the motor protein _____.
COP II
Via the motor protein DYNEIN
In ALS patients, the golgi can become ____, leading to decreased protein production, axonal transport, muscle weakness, and paralysis.
FRAGMENTED
DMD is characterized by a lack of DMD protein which leads to ______ of golgi. This is considered a ____ disorder. Parkinson’s on the other hand is considered a _____ disorder, and is characterized by _____ of the golgi. Mutations in ____ 1,2, and 8 are the cause for Parkinson’s.
Lack of DMD protein leads to DISORGANIZATION of golgi. This is considered a MUSCULAR disorder. Parkinson’s is considered a NEUROLOGICAL disorder, and is characterized by FRAGMENTATION of the golgi. Mutations in RAB 1,2, and 8 are the cause.
Viral RNA from HRV 1 and Hepatitis C can fuse with the golgi, causing it to _____.
FRAGMENT
Staining of _____ ____ is used to identify Lysosomes.
ACID PHOSPHATASE
Primary lysosomes form from vesicles that bud off from the _____, and contain hydrolytic enzymes. These enzymes are marked with _____ which is recognized by specific receptors at the Trans end of the ____.
GOLGI. Marked with M6P
Residual bodies, termed _____, arise in Secondary Lysosomes from undigested material
LIPOFUSCIN
Autophagolysosomes can increase with sublethal cellular injury, but typically _____ with aging.
DECREASE
When hydrolases are deficient or absent from lysosomes, _____ bodies can form from undigested material.
MYELINOID bodies.
Tay-sachs disease is a ganglioside storage disease resultant from a mutation in _____ aminidase. It leads to symptoms such as progressive ____ and ____ deterioration.
HEXOSE aminidase. Leads to progressive MOTOR and MENTAL deterioration.
Nieman-Pick disease affects the CNS and PNS and is characterized by severe neurodegeneration and _______. Cells may appear to have _____ bodies. This disease can be caused by mutations in sphingomyelinase or Nieman Pick proteins 1 and 2 (the latter leads to accumulation of _____ and sphingolipids.)
Neurodegeneration and HEPATOSPLENOMEGALY. Cells may appear to have ZEBRA bodies. Mutations in NP protein 1 and 2 leads to accumulation of CHOLESTERL and sphingolipids.)
Pompe disease results from a mutation in ____ and leads to accumulation of Glycogen. This can be detrimental in the heart where muscle contraction may cause lysosomes ballooned with glycogen to burst.
alpha-1,4-GLUCOSIDASE
Gaucher disease affects ______, which accumulate glucocerebrosidase. This can lead to ____ disorders and hepatosplenomegaly.
MACROPHAGES. Can lead to BONE disorders.
In I-cell disease, hydrolytic enzymes are not phosphorylated (marked with ____) and are thus not recognized by the appropriate receptor in the trans end of the golgi. Clinical symptoms can include irregular constriction of _____ and eventually hand _____.
marked with M6P. Clinical symptoms can include irregular constriction of METACARPALS and eventually hand CONTRACTURES.
Rheumatoid arthritis results from a greater than normal presence of Lysosomes in _____ membranes. This can lead to joint pain and inflammation.
SYNOVIAL membranes
Silicosis/ asbestosis can lead to necrosis and _____ because the sharp particles cannot be broken down, and instead rupture the phagolysosomes that take them up.
Necrosis and FIBROSIS
