2. Leigh Syndrome – decreased activity in cytochrome c oxidase

3. Brain tissue loss and muscular deficiency

4. terminal

5. Complex II deficiency

6. Short stature, seizures, mild swelling in and around the brain, encephalopathy

7. Not terminal

8. Beriberi

9. Lack of thiamine, vitamin B1

10. Decreased activity in pyruvate dehydrogenase and alpha-ketogluterate dehydrogenase (regulatory enzyme in krebs cycle)

11. Can take thiamine supplements

12. w/out B1, krebs cycle can work as well

13. nervous system disorders.

14. The cytoskeleton regulates

15. cellular mitosis

16. meiosis

17. cell motility (entire cell)

18. Motility and trafficking of proteins, organelles, RNA in the cell

19. Cell-cell adhesion

20. Counterparts in Bacteria – slide 2

21. Microtubules (formed by dimmers of the tubulin monomers) – counterpart in bacteria, FtsZ gene

22. cell division

23. Actin (microfilaments) – counterpart in bacteria, MreB

24. DNA segregation

25. Intermediate Filaments – counterpart in bacteria Crescentin

26. Maintaining shape

27. Properties of Microtubules, Microfilaments, and Intermediate Filaments

28. Microtubules

29. Growing and shrinking end

30. Formed by the dimer tubulin monomer

31. Function

32. Organized into structure that allows for a specific motility

33. Axoenemal

34. Whip-like movement such as sperm cells.

35. Make up much of the cytoplasm to make a dense environment

36. Chromosomal movement

37. Microfilaments/Actin filaments

38. Actin is smallest

39. Makes microfilaments

40. Link g-actin together non-covalently

41. Cell division

42. Amoeboid Movement

43. Cytosol of an amoeba is a gel, or plasma gel. The gel is converted to plasmasol, so it’s more fluid and the movement is more fluid

44. If you have a structure like an amoeba, the cytosol is more gel like than water, so plasma gel. For movement, the gel is converted to plasmasol, so it’s more fluid and the movement is more fluid, which tends to create a force or pressure along membrane as force is mediated

45. Intermediate Filaments – structure and support for cells

46. No polar sides! So very good at structural support system for cells.

47. Structural support

48. Maintain shape

49. Nuclear lamina

50. Just on the inside of nuclear envelope

51. Strengthening of nerve cells

52. Desmin helps maintain muscle cell shape when undergoing mechanical movement

53. Not as dynamic as microfilaments and microtubules

54. Rope-like structures

55. Cytoplasmic filament (slide 3) have concentrated areas in sites called desmosomes, which provide linkage between cells to hold them. Proteins make desmosomes, but the filaments hold the cells together by interacting with desmosomes. They don’t hold directly to cells, but link to one another to hold cells together in a tissue structure.

56. Nuclear lamins (blue area around nucleus)

57. Can help regulate what goes in and out of nucleus

58. (Slide 4) 8-12nm. Begins with assembly of alpha helix monomer, made of four alpha helices with 3 regions that separate the monomer itself. 310-318 amino acids, all of which are hydrophilic. At the end, amino and carboxy terminus, hydrophobic, tend to be regions that associate with other types of proteins.

59. Coiled-coil dimers come together in a staggered position for amino and carboxy terminus do not overlap, and this allows the tetramers to pack together at the heads, through non-covalent bonding. Eight tetramers are brought together and form a rope-like structure.

60. Classes of Intermediate Filaments

61. Specificity of intermediate filaments can help us identify cells and localize the filaments in the cell.

62. Slide 6 is trying to show intermediate filaments at the desmosomes

63. Epidermolysis bullosa simplex

64. K14 gene generates two types of Keratin, class I and II.

65. Any amount of stretching will rip epithelial cells apart and can lead to blistering.

66. Neurofilament diseases

67. Epidermalisis – lysing of the epidermis below cymplex

68. Mutation in keratin – k14 gene – that is responsible for generating two classes of keratin, I and II, when they can’t function they can’t hold selves together, so blistering wounds can be found on the skin.

69. Lou Gerhig’s Disease (ALS – Armyotropic Lateral Sclerosis)

70. NF-H, Peripherin

71. Protein that is mutated as well as neuro fiaments

72. Sometimes not terminal, depends of genetics of person

73. Cardiomyopathies – desmoplakin, which can induce heart failure.

74. Nuclear Lamins is formed by lamin proteins

75. Polarized cell.

76. Found under the nuclear envelope, and separating the chromatin from the envelope.

77. Laminopathies

78. Lipodystrophy

79. Fat cells are abnormally deposited

80. Heart attacks, high BP etc

81. ADLD – Autosomal Dominant Leukodystrophy

82. Loss of myelin mutation in Lamin A

83. Can lead to loss of motor control/paralysis

84. Assembly of microtubules (Slide9)

85. Alpha and beta dimers

86. Oligomer

87. Nucleation site

88. AREAS WHERE MORE DIMERS CAN ADD ON AND LENGTHEN THESE OLIGOMERS into a protofilament.

89. 13 protofilaments form a microtubule when they close

90. once closed, either end can be an end where dimmers come on or off so either end can be plus or minus end.

91. Either end can grow and shrink

92. Line up so plus and minus ends are in same region of the cells, - at nucleus, plus at end of axon where growing takes place.