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1

What is stargardt's disease also known as

fundus flavimaculatus

2

Stargardt's disease is the most common from of this

juvenile macular degeneration

3

Stargardt's disease is the loss of this

central vision between the ages of 6 and 20

4

Stargardt's disease starts as this

presence of small, yellow spots in eye

5

With stargardt's disease vision loss begins this way then quickly does this

slowly
escalates

6

Causes of stargardt's disease

Very strong genetic component
Mutation of the gene ABCA4 (one of 21 genes coding for the retina)

7

Stargardt's disease: 90% of the cases are this

autosomal recessive

8

Stargardt's disease: Specific cause

a dysfunctional protein is made which does not allow normal transport of energy from the photoreceptors centered in the macula lutea

9

Stargardt's disease: Lipofuscin build up can result in this

an imbalance between formation and disposal mechanisms in the eye, and is related to the muation in the ABCA4 gene

10

Stargardt's disease: The gene is also associated with this

vitamin A processing

11

Stargardt's disease: signs and symptoms

blurry/distorted vision
loss of fine focusing
difficulty recognizing familar faces
can lead to blindness
deterioration of light sensitive cells
color vision affected in late stages

12

Stargardt's disease: children with the disease will have difficulty doing this

reading

13

Stargardt's disease: treatment

no cure
little can slow progression
wearing sunglasses may slow progression
avoiding excessive vitamin A and Beta-carotene

14

Stargardt's disease: excessive vitamin A and Beta-carotene do this

promote accumulation of lipofuscin

15

Stargardt's disease: prognosis

No cure
eventually leads to blidness in most cases

16

Stargardt's disease: affects this many Americans, and has a frequency of this in children

25000 Americans
1 in 10000 children

17

Primary sex organs: male, females

testes
ovaries

18

Sperm and ovum are these types of cells and are denoted in this manor

gamates, haploid
(n)

19

Fertilization results in this

zygot

20

Sex chromosomes: male, female

XY
XX

21

The zygot grows through

mitosis

22

These develop in early male embryo

seminiferous tubules
intersitial cells

23

Indifferent gonads are found at this stage

early embryonic

24

This determines if indifferent gonads will form testes or ovaries, and results from this

TDF, testes determining factory.
SRY gene on Y chromosome

25

When do the ovaries develop in the embryo

follicles do not develop until third trimester

26

Testes produce these two things

testosterone
Mullerian inhibition factor (MIF)

27

MIF degenerates this

Paramesonephric (mullerian) duct

28

The paramesonephric (mullerian) duct becomes this in men, and this in woman

Degenerates in men
froms uterus and uterine tubes in females

29

Epididymides, ductus deferentia, ejaculatory ducts are formed from this in the presence of this

mesonephric (wolffian) duct
testosterone

30

This happens to the mesonephric (wolffian) duct in females

degenerates because there is no testosterone