20 - Lecture No: 1 (Cellular organization) Flashcards

1
Q

What is a child deficient in if showing Tay-Sachs disease?

A

HEXA - Beta hexosaminidase A, alpha subunit

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2
Q

What does rER do?

A

site of initial post-transitional modifications and folding

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3
Q

What is the largest organelle in the cell?

A

Nucleus - 10% of cell volume

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4
Q

Peroxisomes

A

Have an important role in fat metabolism

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5
Q

Centomere

A

region that holds the sister chromatids together, site of kinetochore formation

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6
Q

Are lysosomes acidic or basic?

A

Acidic - pH 4.7

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7
Q

Coatomer-coated vesicles?

A

control the traffic flow between the ER and the Golgi

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8
Q

What is the first LSDs characterized?

A

Tay-Sachs disease - It’s caused by the absence of an enzyme that helps break down fatty substances. These fatty substances, called GM2 gangliosides, build up to toxic levels in the child’s brain and affect the function of the nerve cells

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9
Q

What is lysosomal storage disease? and what is the most common?

A

inherited metabolic disease resulting in high amounts of toxic materials in the body’s cells as a result of enzyme deficiencies.
Most common: Gaucher Disease - unable to break down lipids(fats) and carbs (sugars)

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10
Q

what is an important factor in the intrinsic apoptosis pathway?

A

cytochrome c

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11
Q

Outer nuclear membrane

A

it faces the cytoplasm

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12
Q

Rough ER

A

synthesis of proteins heading towards the plasma membrane, lysosomes or secretion

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13
Q

What do plasma cells secrete?

A

Secrete antibodies

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14
Q

What does the outer membrane of the mitochondria contain?

A

Porins - large channel forming proteins

Enzymes - phosphlipase and acetyl coenzyme a

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15
Q

Where are mitochondria mainly found?

A

In cells that need to generate large amounts of energy - striated muscle

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16
Q

sER

A

Has cells for lipid metabolism and cells to secrete steroids, plays a major role in detoxification

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17
Q

Zellweger Syndrome

A
  • Show with Hypotonia (poor muscle tone), skeletal abnormalities, vision loss, hearing loss,
    Mutations in genes required for peroxisome function
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18
Q

What is the NPC made up of?

A

Nucleoporins

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19
Q

Domains of cellular life

A

Bacteria(Prokaryotic cells)
Archaea(Prokaryotic cells)
Eukaryota(Eukaryotic cells)

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20
Q

What is the faulty enzyme in 1-cell disease and what is buliding up?

A

Phosphotranseferase and lysosomal hydrolyses and abscent in lysosomes.

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21
Q

What is the faulty enzyme in Pompe and what is building up?

A

alpha-1.4-glucosidase and gylcogen

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22
Q

Smooth ER

A

Synthesis of lipids and detoxification

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23
Q

What are the three pathways to lysosomal digestion?

A

Phagocytosis, Endocytosis, Autophagy

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24
Q

How is chromatin packaged?

A

Packaged into nucleosomes (complex of DNA and proteins called histones)

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25
G
Initial ribosomal assembly
26
FC
stains pale, DNA loops of 5 chromosomes (13,14,15,21,22), contains rRNA genes
27
What is the faulty enzyme in Tay-Sachs and what is building up?
Beta-hexosaminidase and GM2 ganglioside
28
Telomere
At the ends of the chromosome, has a repeated sequence allowing replication.
29
Myoclonic epilepsy with ragged red fibers?
Due to mutation in the MT-TK tRNA gene | might show: myopathy (muscle weakness), ataxia (uncoordinated), dementia (slowed intelectual function)
30
Example of sEr detoxifying?
Cytochrome P450 - these proteins oxidize steroids, fatty acids, and xenobiotics, and are important for the clearance of various compounds, as well as for hormone synthesis and breakdown.
31
What is autophagy?
Self eating.
32
What causes a Lamin A mutation?
Hutchinson-Gilford Progeria sundrome
33
Replication origin
where DNA replication begins
34
2 faces of the golgi apparatus?
cis golgi network (entry) and trans golgi network (exit)
35
Chromatin
Chromosomes that are in different stages of uncoiling
36
Components of the Nucleus
Nuclear envelope, Nuclear lamina, Nuclear pores, Nucleolus, Nucleoplasm
37
What do pancreatic acinar cells?
Secrete digestive enzymes
38
What would a patient present with if having a LSDs?
normal breath, slower growth, bone and joint deformities, frequent lung infections.
39
3 major functions of the inner membrane?
oxidation reactions - respiratory electron transport chain Synthesize ATP Regulate transport into and . out of matrix
40
What makes ER rough?
Ribosomes
41
What is the faulty enzyme in Gaucher and what is buliding up?
Glucocerebrosidase and glucosylceramide
42
what transport COP-1 coated vesicles do?
Retrograde transport - CGN back to the ER
43
What does each organelle contain?
Set of enzymes and molecules
44
What does mRNA need to bind to rER?
They need a ER signal sequence
45
What do lysosomes do?
intracellular digestion of macromolecules
46
What does the Nucleolus do?
Site of ribosomal production and ribosomal RNA is transcribed.
47
What is in Eukaryotic cells?
Nucleus, Ribosomes, Endoplasmic Reticulum, Golgi, Lysosomes, Mitochondira, Peroxisomes
48
What is the stored in the Matrix?
Calcium and other cations
49
Nuclear Pore Complex (NPC)
Allows transport of molecules between the nucleus and the cytoplasm
50
Inner nuclear membrane
provides structure and is in contact with the nuclear lamina
51
Structure of a chromosome?
Centromere, Telomere, Replication origin
52
What are the proteins found in Mitochondria?
7 - NADH 2 - ATP 3 - Cytochrome c oxidase 1 - Cytochrome b
53
How to transport molecules >9nm?
active transport
54
What does the intermembrane space of the mitochondria contain ?
enzymes that use ATP generated by inner membrane - creatine kinase and adenylate kinase
55
Proteasome-mediated protein degradation?
Degrading proteins without involving lysosomes, NEEDS ATP
56
What transport does COP-2 coated vesicles do?
Anterograde transport - carry new proteins from rER to CGN
57
What are the two types of chromatin?
Euchromatin - E(lightly stained, less chromatin, more transcriptionally active) Heterochromatin - H(dark stain, more chromatin, less transcriptionally active)
58
What are the 3 zones of the Nucleolus?
Fibrillar Center (FC), Fibrillar material (F), Granular material (G)
59
What is the Macromolecular complex?
Octamer (8 histone complex), 2 loops of DNA wrap around the core octamer
60
How to transport ions and molecules <9nm?
diffuse freely through the pore
61
F
Transcription of rRNA genes
62
What is the faulty enzyme in Hurler syndrome MPS 1 and what is building up?
alpha-L-iduronidase and Dermatan sulphate
63
T or F: Nucleosomes are found in only Euchromatin?
False Nucleosomes are found in both Euchromatin and Heterochromatin
64
Nuclear Lamina
Thin sheet like meshwork beneath the inner nuclear membrane
65
Do neurons have rER? What do they do?
Nissl Bodies | Synthesize protein neurotransmitters
66
What do rER in plasma cells do?
Synthesize and secrete antibodies
67
Pericentriolar material
PCM | Does microtubule nucleation and anchoring
68
Centrioles
Production of spindle fibers
69
What makes up the centrosome? | What does the centrosome do?
PCM Aand Centrioles | Organizes microtubules and regulates cell division
70
Proteasome
Uses proteolysis (break peptide bonds)to break down unneeded or damaged proteins
71
Prokaryotic cell
Bacteria and arch bacteria consist of a single compartment surrounded by plasma membrane
72
What are the proteins that make up the NPC?
Nucleoporins
73
Which chromatin is tightly packed? | Which chromatin is loosely packed?
``` Tight = heterochromatin Loosen= euchromatin ```
74
What are abundant in cells that specialize in protein synthesis?
rER
75
Golgi Apparatus functions?
Post transitional modification, sorting, packaging
76
How many enzymes are in lysosomes?
40 types of hydrolysis enzymes