Re:[tips] Smell Cilia

[Mike Wiliams]

The main problem with these studies is the use of a pathology that does 
not exist in nature. The authors: ... the relevance of IFT88 mutations 
to human pathology is unknown. The logic follows the line that, We 
produced a mouse that doesn't have protein IFT88 and this protein is 
necessary for cilia growth.  We discovered that when we give the mouse a 
treatment that increases protein IFT88, they grow cilia.  An IFT88 
protein deficit is not a natural illness.  It was apparently produced by 
a type of selective inbreeding.  It reminds me of the attempts to treat 
scopolamine-induced memory disorder.  A number of medications were 
effective but none panned out as effective with any naturally-occurring 
memory disorder.

I wonder if the hearing and balance systems are poor in these mice.  The 
cilia in these systems are much more important than smell.

Mike Williams


On 9/4/12 1:00 AM, Teaching in the Psychological Sciences (TIPS) digest 
wrote:
 TIPS Digest for Monday, September 03, 2012.

 1. What's That Smell?
 2. What's That Smell: Dogs  Orcas Edition
 3. Re: What's That Smell: Dogs  Orcas Edition
 4. The Effective But Forgotten Benezet Method of K-8 Education

 --

 Subject: What's That Smell?
 From: Michael Palijm...@nyu.edu
 Date: Mon, 3 Sep 2012 08:59:40 -0400
 X-Message-Number: 1

 Some new research involving gene therapy in a mouse model shows
 promise for treating a group of disorders called ciliopathies which are
 dysfunctions of the cilia.  Most psychologists are familiar with cilia
 from the role they play in hearing, seeing, and smell.  The new research
 focuses on how to repair the cilia in mice that have genetically disabled
 olfactory cilia, that is, mice who are born without a sense of smell.
 If such gene therapy is effective in humans, then a number of ciliopathies
 might be cured or significantly improved.

 The popular media has picked up on the story and here is one example
 of their presentation:
 http://www.bbc.co.uk/news/health-19409154

 A pop science presentation on the Science Daily website is available
 here (it provides much more detail and additional links):
 http://www.sciencedaily.com/releases/2012/09/120902143147.htm

 Some of the researchers involved in the study are at the University
 of Michigan and the U of M media office provided this press release:
 http://www.uofmhealth.org/news/archive/201209/smell

 The original research is published in Nature Medicine:
 http://www.nature.com/nm/journal/vaop/ncurrent/full/nm.2860.html

 The reference for the article is:

 Jeremy C McIntyre, Erica E Davis, Ariell Joiner, Corey L Williams,
 I-Chun Tsai, Paul M Jenkins, Dyke P McEwen, Lian Zhang, John
 Escobado, Sophie Thomas, Katarzyna Szymanska, Colin A Johnson,
 Philip L Beales, Eric D Green, James C Mullikin, NISC Comparative
 Sequencing Program, Aniko Sabo, Donna M Muzny, Richard A Gibbs,
 Tania Attié-Bitach, Bradley K Yoder, Randall R Reed, Nicholas Katsanis,
 Jeffrey R Martens. (2012).
 Gene therapy rescues cilia defects and restores olfactory function
 in a mammalian ciliopathy model.
 Nature Medicine, 2012;
 DOI: 10.1038/nm.2860

 I suspect that if this research is successful in humans, then olfactory
 abilities lost to toxins and age might be successfully treated.  It may
 be particularly useful in the elderly who have developed a diminished
 sense of smell.

 -Mike Palij
 New York University
 m...@nyu.edu

 P.S.  One point for the person who can guess which movie the subject
 line is from. ;-)



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Re: [tips] Smell Cilia

[Carol DeVolder]

This if fascinating stuff. I wonder if someone could explain to me
something from the article. A little Googling only served to confuse me
more. The article posits that photoreceptor outer segments have a 9+0
arrangement, which should mean that there are nine pairs of cilia arranged
in a ring-like structure. That doesn't connect to my idea of the outer
segment being a single entity--not paired, and not nine of them per cell.
Granted, I read through the article rather quickly, but if someone could
explain, I'd appreciate it--back channel is fine since others may not be as
fascinated by cilia as I am.
Thanks,
Carol


On Tue, Sep 4, 2012 at 7:59 AM, Michael Palij m...@nyu.edu wrote:

 I don't pretend to be an expert on the ciliopathies (not even on the
 internet) but there are a variety of different types of disorders because
 different types of cilia cells are located through the body..  Consider
 the following review article:

 Armengot Carceller, M et al (2010). Primary ciliary dyskinesia.
 Ciliopathies Acta Otorrinolaringologica - English Edition 2010
 Volume:  61  Issue:  2  Page:  149-159.

 The above is available in PDF form at:

 http://www.elsevier.es/sites/default/files/elsevier/pdf/102/102v61n02a13147310pdf001_2.pdf

 Table 1 identifies different types of cilia and their locations.
 Photoreceptor cilia serve as rods and cones while Kinocilia
 serve in the inner ear.  I don't know but someone can check
 if someone who develops retinitis pigmentosa (an eye disorder)
 also develop hearing problems but at least one source points
 out that the defect that cause retinitis pigmentosa is related
 to the development of Senior–Løken syndrome, a kidney based
 disorder where the cilia suffer the same defect; see:
 http://ezproxy.library.nyu.edu:3659/doi/full/10.1056/NEJMra1010172

 I'm only guessing but I think that reasonable general review of
 cilia and ciliopathy are provided in the following:
 http://jcb.rupress.org/content/180/1/17.long
 and
 http://www.annualreviews.org/doi/abs/10.1146/annurev.genom.7.080505.115610

 One final point, the little tail on sperm is also a cilia cell.

 -Mike Palij
 New York University
 m...@nyu.edu

 -   Original Message ---
 On Tue, 04 Sep 2012 00:52:49 -0700, Mike Wiliams wrote:

 The main problem with these studies is the use of a pathology that does
 not exist in nature. The authors: ... the relevance of IFT88 mutations
 to human pathology is unknown. The logic follows the line that, We
 produced a mouse that doesn't have protein IFT88 and this protein is
 necessary for cilia growth.  We discovered that when we give the mouse a
 treatment that increases protein IFT88, they grow cilia.  An IFT88
 protein deficit is not a natural illness.  It was apparently produced by
 a type of selective inbreeding.  It reminds me of the attempts to treat
 scopolamine-induced memory disorder.  A number of medications were
 effective but none panned out as effective with any naturally-occurring
 memory disorder.

 I wonder if the hearing and balance systems are poor in these mice.  The
 cilia in these systems are much more important than smell.

 Mike Williams

  Subject: What's That Smell?
  From: Michael Palijm...@nyu.edu
  Date: Mon, 3 Sep 2012 08:59:40 -0400
 
  Some new research involving gene therapy in a mouse model shows
  promise for treating a group of disorders called ciliopathies which are
  dysfunctions of the cilia.  Most psychologists are familiar with cilia
  from the role they play in hearing, seeing, and smell.  The new research
  focuses on how to repair the cilia in mice that have genetically disabled
  olfactory cilia, that is, mice who are born without a sense of smell.
  If such gene therapy is effective in humans, then a number of
 ciliopathies
  might be cured or significantly improved.
 
  The popular media has picked up on the story and here is one example
  of their presentation:
  http://www.bbc.co.uk/news/health-19409154
 
  A pop science presentation on the Science Daily website is available
  here (it provides much more detail and additional links):
  http://www.sciencedaily.com/releases/2012/09/120902143147.htm
 
  Some of the researchers involved in the study are at the University
  of Michigan and the U of M media office provided this press release:
  http://www.uofmhealth.org/news/archive/201209/smell
 
  The original research is published in Nature Medicine:
  http://www.nature.com/nm/journal/vaop/ncurrent/full/nm.2860.html
 
  The reference for the article is:
 
  Jeremy C McIntyre, Erica E Davis, Ariell Joiner, Corey L Williams,
  I-Chun Tsai, Paul M Jenkins, Dyke P McEwen, Lian Zhang, John
  Escobado, Sophie Thomas, Katarzyna Szymanska, Colin A Johnson,
  Philip L Beales, Eric D Green, James C Mullikin, NISC Comparative
  Sequencing Program, Aniko Sabo, Donna M Muzny, Richard A Gibbs,
  Tania Attié-Bitach, Bradley K Yoder, Randall R Reed, Nicholas Katsanis,
  Jeffrey R Martens. (2012).
  Gene therapy rescues cilia defects and 

Re: [tips] Smell Cilia

[Carol DeVolder]

Those were both very helpful, Mike, thank you!!!
Carol


On Tue, Sep 4, 2012 at 2:34 PM, Michael Palij m...@nyu.edu wrote:

 For one straighforward description, see:
 http://www.vetmed.vt.edu/education/Curriculum/vm8054/eye/RODCONE.HTM

 Quoting from the description of the second image:

 |The image at the left, a transmission electron micrograph, shows
 |the waist between the inner and outer rod segments. The light
 |sensitive lamellae of the outer segments are obvious, as is the
 |narrow constriction. Note that in the constriction itself, there is
 |an actual modified cilium, complete with basal body and ciliary
 |rootlets. This remarkable structure is proof that the outer segment
 |is really a highly modified cilium, and that the membranous lamellae
 |are extensive ramifications of its plasma membrane. the modification
 |of cilia to perform sensory functions is found in other locations in
 |mammals: the olfactory cells of the nose are another example of
 |a highly-specialized neuron with greatly-modified cilia devoted to
 |sense perception rather than movement of fluid.

 For a tradition review of the subject, see:

 http://pharmacology.case.edu/department/faculty/primary/pages/Palczewski/articles/Progress%20in%20Retinal%20and%20Eye%20Research.pdf

 A search for cilia in the article will locate relevant sections.

 -Mike Palij
 New York University
 m...@nyu.edu


   Original Message 
 On Tue, 04 Sep 2012 10:12:00 -0700, Carol DeVolder

 This if fascinating stuff. I wonder if someone could explain to me
 something from the article. A little Googling only served to confuse me
 more. The article posits that photoreceptor outer segments have a 9+0
 arrangement, which should mean that there are nine pairs of cilia arranged
 in a ring-like structure. That doesn't connect to my idea of the outer
 segment being a single entity--not paired, and not nine of them per cell.
 Granted, I read through the article rather quickly, but if someone could
 explain, I'd appreciate it--back channel is fine since others may not be as
 fascinated by cilia as I am.
 Thanks,
 Carol


 On Tue, Sep 4, 2012 at 7:59 AM, Michael Palij m...@nyu.edu wrote:

  I don't pretend to be an expert on the ciliopathies (not even on the
  internet) but there are a variety of different types of disorders because
  different types of cilia cells are located through the body..  Consider
  the following review article:
 
  Armengot Carceller, M et al (2010). Primary ciliary dyskinesia.
  Ciliopathies Acta Otorrinolaringologica - English Edition 2010
  Volume:  61  Issue:  2  Page:  149-159.
 
  The above is available in PDF form at:
 
 
 http://www.elsevier.es/sites/default/files/elsevier/pdf/102/102v61n02a13147310pdf001_2.pdf
 
  Table 1 identifies different types of cilia and their locations.
  Photoreceptor cilia serve as rods and cones while Kinocilia
  serve in the inner ear.  I don't know but someone can check
  if someone who develops retinitis pigmentosa (an eye disorder)
  also develop hearing problems but at least one source points
  out that the defect that cause retinitis pigmentosa is related
  to the development of Senior–Løken syndrome, a kidney based
  disorder where the cilia suffer the same defect; see:
  http://ezproxy.library.nyu.edu:3659/doi/full/10.1056/NEJMra1010172
 
  I'm only guessing but I think that reasonable general review of
  cilia and ciliopathy are provided in the following:
  http://jcb.rupress.org/content/180/1/17.long
  and
 
 http://www.annualreviews.org/doi/abs/10.1146/annurev.genom.7.080505.115610
 
  One final point, the little tail on sperm is also a cilia cell.
 
  -Mike Palij
  New York University
  m...@nyu.edu
 
  -   Original Message ---
  On Tue, 04 Sep 2012 00:52:49 -0700, Mike Wiliams wrote:
 
  The main problem with these studies is the use of a pathology that does
  not exist in nature. The authors: ... the relevance of IFT88 mutations
  to human pathology is unknown. The logic follows the line that, We
  produced a mouse that doesn't have protein IFT88 and this protein is
  necessary for cilia growth.  We discovered that when we give the mouse a
  treatment that increases protein IFT88, they grow cilia.  An IFT88
  protein deficit is not a natural illness.  It was apparently produced by
  a type of selective inbreeding.  It reminds me of the attempts to treat
  scopolamine-induced memory disorder.  A number of medications were
  effective but none panned out as effective with any naturally-occurring
  memory disorder.
 
  I wonder if the hearing and balance systems are poor in these mice.  The
  cilia in these systems are much more important than smell.
 
  Mike Williams
 
   Subject: What's That Smell?
   From: Michael Palijm...@nyu.edu
   Date: Mon, 3 Sep 2012 08:59:40 -0400
  
   Some new research involving gene therapy in a mouse model shows
   promise for treating a group of disorders called ciliopathies which are
   dysfunctions of the cilia.  Most