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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