Biology 610: Current Topics in Cell and Molecular Biology

                                                                                                                        

Fall Semester 2003 Molecular Biology 610:  Molecular and Cell Biology of Disease

(Please note meeting room, date and time of lectures)

 

 

Instructor: Roger Davis, LS307, rdavis@sunstroke.sdsu.edu; 619-594-7936).

 

The goal of this course is to provide students with insights into how modern cell and molecular biology is used to gain and understanding of disease processes and/or the development of therapies.  Each week the course will concentrate on one related topic.  On Mondays (except November 18- see below) outside experts will come a give the first lecture (didactic and up to 1.5 hours).  This lecture will provide students with both a review of the current literature as well as the presenter’s own view of where the field is headed.

The second class meeting will be a class discussion in which two research publications (provided via the class website in the form of a PDF file) are discussed by the students and led by the instructor.  Each student is responsible for having read the paper and being prepared to describe to the class the salient points of the paper, which include:

1)    understanding all data presented in figures and tables

2)    understanding what conclusions were offered by the authors

3)    understanding how these conclusions are (or are not) supported by the data

4)    being prepared to propose alternative conclusions and experiments to further examine the major issues of the papers

Grades will be calculated as 50% class participation and 50% final exam.  Each person can only miss one class meeting!

Oct 22  Sandy Bernstein will finish discussion of first part of class.  Class should begin studying papers for week of Oct 27.

Oct 27 Stanley Maloy, Professor of Biology, SDSU; Topic: “Infectious bacteria and human disease”

Nov 3 Linda Curtiss, Member, Scripps Research Institute; Topic: “Biology of bone marrow transplantation and its therapeutic importance”

Nov 10  Simon Hui, Research Assistant Professor of Biology, SDSU; Topic: “Nuclear receptors”

TUESDAY, Nov 18 (LS101) Chris Glass, Professor of Cell and Molecular Medicine, UCSD; Topic: “PPARs and metabolic diseases”

Nov 24   Mark Sussman, Professor of Biology, SDSU; Topic:“Cardiac myocyte development and regeneration”

Dec 1 Jim Paterniti, Senior Director, Pharmacology, Amylin Pharmaceuticals, Inc.; Topic:  “Diabetes, obesity and energy metabolism”

Dec  8  Joseph Witztum, Professor of Medicine, UCSD; Topic:“innate immunity and atherosclerosis”

Dec 10  Final examination

___________________________________________________________

Topic 1, day 1 presented by Stanley Maloy on Nov 3-5

1. Bacteria-host interactions—normal flora and symbiosis

a.   Host immunity

b.   Other host functions

2. Bacteria-host interactions gone array:

a.   What is a pathogen? Microbe vs host

b.   What is a virulence gene? Molecular Koch’s postulates

c.   Evolution of pathogenesis

d.   How can you identify bacterial genes  required for virulence

Two papers that emphasize some of these concepts are attached

Bacteria-host interactions—normal flora and symbiosis (Bacteroides PDF file)

How did they demonstrate that the observed host response was elicited by Bacteroides?

Why did they focus on Bacteroides? Do all commensal bacteria stimulate a similar response?

How might bacterial cells promote this host response?

How did they visualize the microvasculature development in the small intestine?

Are there better ways this could be done?

Why did they focus on Paneth cells?

What are angiogenins? 

Not as simple a question as it sounds ... see Nat Immunol. 2003 4: 269]

Why is this a “symbiosis”?

Evolution of pathogenesis (Listeria pdf file)

What is the evidence that internalin is specifically involved in the transit of Listeria across the gut?

Does internalin affect the systemic infection?

What is the evidence that E-cadherin is the host receptor?

What is the difference in E-cadherin between humans, guinea pigs, and mice?hat is the proposed role for inlB and why might this be required

 

Topic 2, presented by Simon Hui on Nov 10-12

Nuclear Receptors Review 1 and Co-activators/repressors

Pick the BEST answer in each

1)  LXR binds to a DR4 site on the CYP7A1 promoter.  Which of the following sequences are may describe the LXR binding site?

A)  AGGTCATAGAAGGTCA

B)  AGGTCATAGAGGTCA

C)  AGGTCATAGATGACCT

D) AGGTCAXTGACCT

E) AGGTCATAGAAGTTC

2)  Which of the following site on a nuclear receptor is responsible for binding to NCOR?

A)  AF1

B)  AF2

C)  AF3

D) H3

E) A/B

3)  Which portion of a PPARg is likely to be similar to that of PPARa?

A)  The ligand binding domain

B)  The AF2 domain

C)  The DNA binding domain

D) A and B

E) B and C

4)  What best describes the LXXLL domain?

A)  It's the charged part of the AF2 domain responsible for binding a co-activator

B)  It's the hydrophobic part of a co-activator responsible for binding to the AF2 domain.

C) It's the hydrophobic part of a co-activator responsible for binding to DNA

D) It's the hydrophilic part of a co-activator responsible for binding to the AF1 domain.

E) It's the portion of the DNA binding domain of a nuclear receptor.

5)  Deacetylated histones tend to

A) Be associated with active transcription

B) Be associated with less active transcription

C) Be associated with less accessible DNA

D) A and C

E) B and C

6)  Which of the following proteins binds to the TATA box with high affinity (<10-7 M?

A) TBP

B) Polymerase III

C) TFIID

D) Polymerase II

E) B and D

7)  Which of the following factors represses transcription but has no DNA binding site?

A) HNF4

B) LXR

C) FXR

D) SHP

E) CAR

8)  FOX and JUN bind to which DNA element?

A) IR1

B) DR7

C) AP1

D) IR

E) all of the above

Topic 3 presented by Chris Glass Professor of Cell and Molecular Medicine, UCSD on TUESDAY, Nov 18 (LS101)

 “PPARs and metabolic diseases”

Paper 1 Li PPARg and athero.pdf and paper 2 PPARd and athero.pdf

Questions for discussion:

Discussion questions for Li, et al., 2000 and Lee, et al., 2003

1.  What are the major physiological roles of each of the PPARs?

(PPARa is highly expressed in liver and heart and plays an important role in fatty acid metabolism and energy homeostasis.  PPARa is the molecular target of the fibrate class of drugs that raise HDL and lower VLDL in the circulation.  PPARg is most highly expressed in adipose tissue and is required for fat cell development.  PPARg also regulated glucose and fatty acid metabolism and is the target of the TZD class of drugs that improve insulin resistance in patients with type 2 diabetes mellitus.  The physiologic roles of PPARd have not as yet been clearly established, but recent  studies suggest roles in energy homeostasis.  Specific, high affinity ligands for PPARd have recently been developed and should allow the pharmacology of PPARd to be explored.)

2.  What is the potential significance of PPARg expression in macrophages with respect to treatment of diabetes and the development of atherosclerosis?

(The discovery that PPARg is expressed in macrophages raised the possibility that it might have additional functions beyond regulation of adipogenesis and regulation of glucose homeostasis.  The goals of treatment of type 2 diabetes are to lower glucose levels and symptoms of hyperglycemia in the short term and inhibit the development of complication in the long term.  Diabetic patients are at markedly increased risk of development of atherosclerosis and its clinical complications.  If the activation of PPARg in macrophages by TZDs had an adverse effect on atherosclerosis, it would not be a desirable drug for use in diabetics despite its ability to lower circulating glucose levels.  Conversely, if activation of PPARg inhibited the development of atherosclerosis, it would be predicted to decrease atherosclerotic complications in diabetic patients.)

3.  Why might activation of PPARg promote the development of atherosclerosis?

(Some of the initial studies of PPARg action in macrophages led to the discovery that it can induce the expression of CD36, a macrophage scavenger receptor  that  can mediate the uptake of atherogenic forms of LDL.  These observations raised the question of whether  PPARg might activate other atherogenic genes.)

4.  Why might activation of PPARg inhibit the development of atherosclerosis? 

(First, TZDs are insulin sensitizers and improve glucose homeostasis.  This in itself may be anti-atherogenic.  Second, studies of PPARg action in the macrophage indicate that it can inhibit inflammatory response genes, including inducible nitric oxide synthase and matrix metalloproteinase 9 (MMP9).  Atherosclerosis is a chronic inflammatory disease, in which the expression of genes that promote influx and activation of monocyte/macrophages and lymphocytes is thought to play a critical role in disease progression.  Inhibition of the expression of these genes by PPARg agonists would be predicted to be antiatherogenic.    Third, recent studies have suggested that PPARa and PPARg can induce the expression of the liver X receptor alpha (LXRa).  LXRa is a nuclear receptor that is activated by oxysterols and acts in a feed-forward regulatory pathway to lower cellular cholesterol levels.  LXRa activates genes such as the ABCA1 transporter that mediates transfer of cellular cholesterol to extracellular acceptors.  Activation of LXRa expression by PPARa and PPARg agonists is not consistently observed, however, and the importance of this pathway remains to be established.)

5.  How do you interpret the differential effects of PPARg agonists on the development of  atherosclerosis in male and female mice?

(These observations suggest that there is a sexual dimorphism that alters the balance of atherogenic and antiatherogenic effects of PPARg agonists in male and female animals. The basis for this effect has not been established.  It does not appear to translate to human subjects, as women are slightly more responsive to TZDs with respect to effects on glucose levels than are men.  The findings in mice are of interest because they suggest that there are regulatory mechanisms that can act to inhibit or counteract PPARg activity.)

6.  To what extent do the findings in Li et al. support local as opposed to metabolic roles of PPARg action in inhibiting the development of atherosclerosis in male mice?

Mice fed the high fat/high cholesterol diet became moderately obese and insulin resistant.  Treatment with the PPARg agonists improved insulin resistance and resulted in decreased levels of inflammatory gene expression in the artery wall.  Thus, the observed results could be due to either local or metabolic actions of PPARg agonists, or both.  The findings in Li et al were subsequently reproduced by three other laboratories.  In one study, a hypercholesterolemic diet was used that did not cause obesity or insulin resistance, suggesting antiatherogenic activities independent of glucose lowering.

7.  How could you directly test whether  activation of  PPARg expression in macrophages is essential for antiatherogenic activities of TZDs?

The general approach to this type of question is to lethally irradiate an atherosclerosis-prone strain of mice (e.g., LDLR KO) and reconstitute with wild type or PPARg knockout bone marrow progenitor cells.  The resulting mice can then be fed an atherosclerotic diet with or without drug and the extent of atherosclerosis measured.  This type of experiment has been done and mice reconstituted with PPARg KO bone marrow get more atherosclerosis than mice reconstituted with wild type bone marrow.  These studies demonstrate that PPARg plays an antiatherogenic role in macrophages.

8.  How might these findings be translated to humans?

9.  The studies reported in Lee et al suggest that PPARd and PPARg regulate different sets of target genes.  The DNA binding domains of PPARd and PPARg are very similar.  How might different target gene regulation be achieved? 

One potential explanation for this would be differential recruitment of coactivators and corepressors.  In the case of MCP-1, PPARd appears to be able to selectively inhibit  its expression due to its selective ability to act as a sink for Bcl6.  The binding of agonists to PPARd are proposed to cause displacement of Bcl6 and its redistribution to the MCP-1 promoter.

10.  How might the redistribution model of inhibition  of MCP-1 be tested further?

One approach would be to use Bcl6-deficient cells.   PPARd agonists should have no effect on MCP-1 expression in these cells.

Another approach would be to perform chromatin immunoprecipitation studies of the MCP-1 promoter.  Bcl6 should be observed on the promoter following addition of a PPARd agonist. 

11.  Atherosclerosis was more severe in LDLR KO animals transplanted with wild type bone marrow progenitor cells that PPARd-KO bone marrow progenitor cells.  Given the effects of PPARd agonists on MCP-1 expression, what do these results imply about the availability of endogenous ligands for PPARd in lesions?

These results suggest that PPARd is unliganded in atherosclerotic lesions, otherwise Bcl6 would have redistributed to the MCP-1 reporter gene.  This is somewhat surprising, because PPARd has been shown to be activated in macrophages by the lipoprotein lipase-dependent hydrolysis of triglycerides in VLDL.  This bears looking into further.

12.  How would you test the hypothesis that atherosclerosis is decreased in LDLR-KO mice due to decreased expression of MCP-1?

Not an easy experiment.   It would require developing double knockouts of  MCP-1 and PPARd and using bone marrow cells from these animals for transplantation studies.  Mice transplanted with MCP-1 KO cells should already be relatively resistant to atherosclerosis, but adding the PPARd KO should no longer result in a further decrease.

13.  Explain how the present studies demonstrate the concept that the phenotype resulting from the lack of a nuclear receptor can be quite different than the phenotype resulting from lack of a ligand. 

14.  Do you think that  activation of PPARd by a synthetic ligand will inhibit the development of atherosclerosis?

Topic 4 presented by Mark Sussman, Ph.D. on November 24, 2003

1)    Paper#1 (Cell, Vol. 114, 763–776, September 19, 2003, Adult Cardiac Stem Cells Are Multipotent and Support Myocardial Regeneration-) and Paper #2 (Nat Med. 2003 Sep;9(9):1195-201. Mesenchymal stem cells modified with Akt prevent remodeling and restore performance of infarcted hearts.  Mangi AA, Noiseux N, Kong D, He H, Rezvani M, Ingwall JS, Dzau VJ.-What are stem cells? Are there differences between a “cardiac stem cell” versus a “hematopoeitic” stem cell?

2)    Where do stem cells live in the heart?

3)    Is the heart a “terminally differentiated” organ? What is the meaning of “self renewal”

4)    What are the problems with stem cell transfer for cardiac regeneration?

5)    How would you select for and enrich the stem cell population?

6)    Can genetic manipulation be helpful for stem cell therapy?

7)    How does Akt potentiate stem cell activity?

8)    Are stem cells capable of regenerating all the tissues necessary to rebuild the damaged heart?

9)    Which is better: using donated stem cells or recruiting endogenous ones for cardiac repair?

10) Why are the resident stem cells not capable of repairing the heart after a heart attack?

11) Do stem cells have a finite lifespan? How many times can they replicate?

12) Why do stem cells behave differently in vitro than in vivo? How do these differences manifest themselves?

13) What are current limitations of stem cell treatment that need to be overcome for this to be a clinically useful therapy for treatment of heart disease?

14) Are you convinced that hearts can be repaired by stem cell transfer?

Topic 5 presented by Linda K. Curtiss, Ph.D. on December 1, 2003

Bone marrow transplantation

 

There will be two papers discussed.  Study questions will be available by the beginning of class.  Paper 1 (JCI96.pdf) describes the transfer of apo E into mice using hematopoietic stem cells (HSC) and paper 2 (PON1 for 610.pdf) describes the delivery of a paraoxonase1 transgene into mice using HSCs.

Study questions

 

  1. Why do LDLr-deficient (LDLr-/-) mice have high levels of plasma low density lipoprotein (LDL)?
  2. Why do apo-E-deficient (apoE-/-) mice have high levels of plasma very low density lipoproteins (VLDL) and intermediate density lipoproteins (IDL) and low levels of plasma high density lipoproteins (HDL)?
  3. What is the red (oil red O) staining material in the intima of the aortic sinus and/or aortic valve lesions?
  4. Different bone marrow-derived cells participate in innate versus adaptive immunity.  Name a cell of the innate immune system and name a cell of the adaptive immune system.
  5. List at least three methods by which successful reconstitution of irradiated and bone marrow transplanted mice can be identified.
  6. Plasma contains lipoproteins that are considered proatherogenic including LDL and VLDL.  Plasma also contains the anti-atherogenic lipoprotein, HDL.  Name two biochemical methods that can be used to separate and identify the cholesterol distribution among these proatherogenic and anti-atherogenic plasma lipoproteins. 
  7. There are two methods that are commonly used in research laboratories to document or quantitate the extent of atherosclerosis in mice.  Name these two methods.  Identify common as well as distinguishing properties of these two measurements.  Name another method you could use to quantitate atherosclerosis in mice.
  8. If you could identify only bone marrow-derived cells and you examined multiple tissues within an animal, list the organs and tissues that would contain marrow-derived cells.
  9. In the apoE-/- bone marrow transplantation studies, the amount of apoE produced by the macrophages appeared to be sufficient for altering the plasma lipoprotein profiles as well as reducing the extent of disease.  How would you go about identifying how much apoE must be present in plasma to alter plasma lipoprotein cholesterol levels and to reduce the extent of atherosclerotic vascular disease?
  10. Can bone marrow transplantation be considered a method of permanent gene therapy?
  11. Foam cell formation is a hallmark characteristic of fatty streak lesions of atherosclerosis.  Both LDL and oxidized LDL can contribute to foam cell formation, which is  characterized by cholesteryl ester accumulation within macrophages.  Is there a difference in the efficiency with which these two LDLs contribute to foam cell formation, and what factors might account for this difference?
  12. Paraoxonase 1 is a glycoprotein made in the liver by hepatocytes.  In vitro this enzyme has been shown to be capable of enzymatically detoxifying organophosphorous pesticides and neurotoxins.  These are exogenous substrates.  Name some endogenous substrates of this enzyme.
  13. At least two different research laboratories have reported the generation and characterization of mice that overexpress PON1, i.e., they have a PON1 transgene.  Why would overexpression of PON1 be expected to contribute to reduced atherosclerosis in apoE-deficient (apoE-/-) mice that also express a PON1 transgene?
  14. Most of the assays examining expression of PON1 in the macrophages of the founder transgenic animals used elicited macrophages, i.e., macrophages that were collected from the peritoneal cavity 48 hr after injection of thioglycolate.  Why was this procedure used?
  15. Why was PON 1 activity identified in the plasma of the non-transgenic littermates of the PON1 Tg mice?
  16. In contrast to the macrophage-specific PON1 transgenic animals, the plasma PON1 activity of the bone marrow transplanted LDLr-/- mice did not differ from that of LDLr-/- mice transplanted with bone marrow from nontransgenic littermates.  Why?
  17. Bone marrow transplantation of macrophage-specific expression of PON1 reduced lesion areas of LDLr-/- mice.  However, bone marrow transplantation of the PON1 transgene into LDLr-/- mice did not alter plasma levels of cholesterol.  This suggests that protection against atherosclerosis by macrophage-specific PON1 did not involve a reduction in plasma cholesterol.  What other mechanism(s) could be involved in the atheroprotective activity of macrophages specific-PON1 expression?
  18. Why were the bone marrow-derived cells from the PON1 BMT mice cultured in L cell conditioned medium before they were examined for cytokine and receptor gene expression by real time PCR?
  19. PON1 expression by macrophages may contribute to reduced oxidation of LDL or enhanced protection of HDL.  How would you test this experimentally?
  20. List 5 other diseases that might be cured by permanent gene transfer via bone marrow transplantation.

Topic 6 presented by Joseph Witztum, MD, Professor of Medicine, UCSD, “Innate immunity and atherosclerosis”

Study questions for two papers (paper 1 immunization) and (paper 2, innate vs acquired immunity)

How does innate and acquired immunity differ?

What components mediate innate immunity?

What components mediate innate immunity?

Why should "immunity" be involved in a chronic disease that essentially

represents the excess accumulation of cholesterol in the artery?

What components of immunity are found in the artery wall?

What is the evidence that manipulations of innate or acquired immunity

affect atherogenesis?

Innate immunity is for the most part genetically determined, if so, why

should this be invoved in atherogenesis, a disease that occurs later in

life, eg beyound the reproductive period?

What are natural antibodies?

What are "pattern recognition receptors" (PRR) and what are "pathogen

associated molecular patterns"(PAMP)?

What are some examples of PRRs and PAMPS?

Is it good or bad to have an immune response involved in atherogenesis?

Could one develop a "vaccine" to prevent or delay the development of atherosclerosis?

Topic 7 presented by Roger Davis, Ph.D., SREBPs, regulators of energy metabolism        Please read review of SREBP-

1)    Which of the following forms of SREBP is primarily involved in regulating genes involved in fatty acid metabolism and also contains an AF2 domain that has a lower affinity for co-activators?

A. SREBP1a

B. SREBP1b

C. SREBP1c

D. SREBP2a

E. SREBP2b

 

2)    Which of the following is a trans-membrane protein in the endoplasmic reticulum?

A)   INSIG-1

B)   SREBP-1c

C)   SCAP

D)   SREBP2

E)    all of the above

 

3) A cell displaying cholesterol auxotrophy is most likely to have mutations that block the function of which gene(s)?

A.      S2P

B.   SCAP

C.   INSIG-1

D.   All of the above

E.    A and B

 

4)    Mature (nuclear) SREBP1a is rapidly degraded in cells by

A.    Lysosomes

B.    proteasomes

C.    Trypsin

D.   The mitochondria

E.    The peroxisomes

 

5)     Which of the following genes display a transcriptional activation by SREBP?

A)   glucokinase

B)   Fatty acid synthase

C)   CYP7A1

D)   A and B

E)    Band C

 

6)    Which end of SREBP binds to the which end of SCAP?

A)   N to N

B)   N to C

C)   C to C

D)   None of the above