Step 1: Cell Components

Phosphorylates the nuclear lamina during prophase of mitosis, which initiates nuclear disassembly into small vesicles

Granular zone-periphery ribosomal precusors
Fibrillar zone- ribonuclear protein fibrils
Fibrillar center- DNA not transcrbied

synthesis for proteins destined for the nucleus, peroxisome, and mitochondria

synthesis of secretory proteins, membrane proteins, and lysosomal enzymes

into cisternae
- studded with ribosomes on the cytoplasmic face
- found next to the nucleus and is prominent within cells that are specialized for secretion (pancreatic acinar cells)

Detoxification reactions

1) Steroid synthesis
2) Hydroxylation - cytochrome P450 (flavoprotein and nonheme iron protein)
3) Conjugation- transfer of a polar group to the toxic water insoluble compound

in the SER
-lipolysis releases fatty acids from triglyceride
- also the site where lipoproteins are assembled

Sarcoplasmic Reticulum
- releases and sequesters calcium ions

organized into cisternae- assembled into stacks
cis and trans face

1) I-cell disease
2) Hyperproinsulinemia

characterized by elevated levels of proinsulin in the serum due to the failure of a peptidase to cleave proinsulin to insulin and C-peptide in the Golgi apparatus

*the clinical manifesations are similar to those seen in patients with non insulin dependent diabetes (DM type II)

A deficiency in N-acetylglucosamine-phosphotransferase results in I- cell disease
*Phosphorylation of mannose in glycoproteins targets proteins to lysosomes
*Phosphate is added in 2 steps: N-acetylglucosamine-phosphotransferase and N-acetylglucosaminidases

*characterized by huge inclusion bodies in cells caused by accumulation of undegraded glucoconjugates in lysosomes missing the hydrolyases that normally degrade these macromolecules

- nucleases - DNA and RNA
- lipases - lipids
- glycosidases- glycoproteins
-proteaes and peptidases - proteins
- phopsphates

contain a number of enzymes that transfer hydrogen atoms from oganic substrates to molecular oxygen with the formation of hydrogen peroxide

Then the enzyme CATALASE - degrades hydrogen peroxide
* synthesis and degradation of H2O2

*beta oxidation of long chain fatty acids (down to 10 C - then moves to mitochondria)
*Phopholipid exchange
*Bile Acid synthesis

1) Zellweger (cerebrohelpatorenal) syndrome
2) Neonatal adrenoleukodystrophy
3) Infantile Refsum disease
4) Hyperpipecolatemia

oxidize many of the substrates in the cell (pyruvate, aa, and fatty acids) generating NADH and FADH2 for the ETC

formed from a protein dimer of alpha and beta tubulin
* 2 accessory proteins: tau and MAP (microtubules associated protein)
**tau- stabilizes microtubules (abundant in neurons and CNS)

-chromosomal movement in meiosis and mitosis
-spindle formation
- intracellular vesicle and organelle transport (dynein{-end} and kinesin {+end})
- ciliary and flagellar movement

characterized by a defect in microtuble polymerization. This leads to defects in cytoplasmic granules including:
- delayed fusion of phagosomes with lysosomes in leukocytes, thus preventing phagocytosis of bacteria
- increased fusion of melanosomes in melanocytes, leading to albinism
-Granular defects in natural killer cells and platelts

Tight junctions = zonula occludens (fusion of two membranes)

Adherent junctions = Zonular adherens

Desmosomes = macular adhereens

Gap junctions = nexus
* made of connexons forming hydrophilic channels

Absent or aberrant dynein arms are found in the cilia and flagella of individuals suffering with this

characterized by chronic sinusitis and bronchiectasis(dilation and destruction of the bronchial tree), as well as infertility and, in some cases situs inversus (inverted position of the internal organs)