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

On Tue, Sep 4, 2012 at 2:34 PM, Michael Palij <[email protected]> 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
> [email protected]
>
>
> ------------  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 <[email protected]> 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
> > [email protected]
> >
> > ---------   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 Palij<[email protected]>
> > > 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 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.
>
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-- 
Carol DeVolder, Ph.D.
Professor of Psychology
St. Ambrose University
518 West Locust Street
Davenport, Iowa  52803
563-333-6482

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