BioMedNet News and Comment

Pazourg

Description

Help FeedbackResearch Journal News & Books & Science WebReviewsTools Collection Comment Labware Jobs LinksLatest Today's Magazine Conference Journal Special Sitenews.bmn.com CommentaryUpdates News HMS Beagle Reporter Scan Report Search My Exit ASCB 2000 - Day 3 - Tuesday 12 December 2000BMNReport: ASCB 2000Conference Reporter The American SocietyDefective cilia underlie killer kidney diseasefor Cell Biology Information Investigator: Greg PazourContents Latest ConferenceTuesday Dec 12th, 2000 Future Conferences Day: 1 2 3 4 Previous by Dan FerberDay 3 Reports:Conferences(Investigator's name) About This Section Defective cilia on urine-collecting ductsSPARring over memory How To Cite Us underlie a devastating kidney disorder called fo rmatio n(Morgan Sheng)autosomal recessive polycystic kidney disease. Editorial BoardKey part of immune systemThe results offer the first real insights into the Staffsame in plants andcause of the disorder, which afflicts one in vertebrates(Ruslan Medzhitov)10,000 children and kills them within monthsQuick Site SearchDefective cilia underlie killerof their birth.kidney diseaseS(Greg Pazour)Cilia project like antennae from most types ofPhosphate-poor andcells in the human body, but their function is gene-rich: how cells make dueAdvanced site search(Erin O'Shea)known in just a few, says Greg Pazour of theMany weak switches makeUniversity of Massachusetts Medical School in one good switch(Tony Pawson)Worcester ...

Informations

• Publié par: Emme
• Nombre de lectures: 26
• Langue: English

Extrait

Help

Feedback

Research

Tools

Reviews

Journal

Collection

News &

Comment

Books &

Labware

Science

Jobs

Web

Links

news.bmn.com

Latest

Updates

Today's

News

Magazine

HMS Beagle

Conference

Reporter

Commentary

Journal

Scan

Special

Report

Site

Search

My

BMN

Exit

Conference Reporter

Information

Latest Conference

Future Conferences

Previous

Conferences

About This Section

How To Cite Us

Editorial Board

Staff

Quick Site Search

S

Advanced site search

ASCB 2000 - Day 3 - Tuesday 12 December 2000

Report:

Defective cilia underlie killer kidney disease

Investigator: Greg Pazour

Tuesday Dec 12th, 2000

by Dan Ferber

Defective cilia on urine-collecting ducts

underlie a devastating kidney disorder called

autosomal recessive polycystic kidney disease.

The results offer the first real insights into the

cause of the disorder, which afflicts one in

10,000 children and kills them within months

of their birth.

Cilia project like antennae from most types of

cells in the human body, but their function is

known in just a few, says Greg Pazour of the

University of Massachusetts Medical School in

Worcester. The best-known cilia in the human

body are those that sweep particles and mucus

along the trachea and the specialized cilia that

help sperm swim. But many other cell types,

including brain, kidney and some liver cells, have

a shorter cilium, called a primary cilium, that

can't move at all.

"They've been known for ages, but nobody really

knows what they do," Pazour says.

To find out, Pazour and his colleagues have been

focusing on a single-celled green alga called

Chlamydomonas

, which contains a pair of

modified cilia called flagella that help it swim.

The basic structure of the algal flagella is

virtually identical to the structure of human cilia,

and it's much easier to use genetic techniques in

the alga to spot new genes and proteins that help

cells build and operate flagella and cilia, Pazour

points out.

In earlier work, researchers had shown that cilia

in those algae are built and maintained in part by

ASCB 2000

The American Society

for Cell Biology

Contents

Day:

1

2

3

4

Day 3 Reports:

(Investigator's name)

SPARring over memory

formation

(Morgan Sheng)

Key part of immune system

same in plants and

vertebrates

(Ruslan Medzhitov)

Defective cilia underlie killer

kidney disease

(Greg Pazour)

Phosphate-poor and

gene-rich: how cells make due

(Erin O'Shea)

Many weak switches make

one good switch

(Tony Pawson)

Pathogens dress up in sheep's

clothing

(Pamela Bjorkman)

New dialects of plant cell

communication

(Joanne Chory and Jennifer

Fletcher)

How the prostate restrains

cancer cells

(Arian Mosbascher)

Tracking RNA in oocytes

(Byeong Cha)

Day 3 Profiles:

Erin O'Shea

Pamela Silver

View all Profiles

ASCB Site

Printer ready version

E-mail article to a friend

a large conglomerate of about 17 proteins that

move up the flagellum, including one called

IFT88. They had also created a collection of

non-swimming mutants of the cells, many of

which have defective cilia. To find out what

IFT88 was doing in the algae, the Massachusetts

team isolated the gene that encoded it, and then

screened the mutant collection to see if any of

them had mutations in the gene.

One mutant did. It turned out that cells with

mutations in the IFT88 gene had no flagella at

all. That meant that the protein was needed to

assemble flagella, Pazour says.

What's more, the protein closely resembled a

protein called Tg737 lacking in mice that develop

symptoms of ARPKD. To nail down the

connection, Pazour's team looked at the kidneys

of the four-day-old mice, and found that cells in

urine collecting ducts had cilia that were only 1

micron long - less than one-third the length of

cilia in normal mice. Their work was published in

November in the

Journal of Cell Biology

.

"We think [Tg737] is required for ciliary

assembly," Pazour says. Next, the researchers

want to know what the cilia are doing in the

kidney. "That's the million-dollar question,"

Pazour says.

"Not only is it cool work, but you've got a very

critical biological entity that depends on [the

protein]," says Kent Hill of University of

California, Los Angeles.

The cilia are most likely acting to sense

mechanical pressure caused by fluid flow or salt

levels, says Bradley Yoder of the University of

Alabama, Birmingham.

In related work presented today, Yoder and his

colleagues analyzed the same line of mice and

showed that they develop liver and kidney

defects like children with autosomal recessive

polycystic kidney disease. Overall, the collection

of symptoms indicates that Tg737 is needed to

build and maintain cilia in kidneys, Yoder says.

Printer ready version

E-mail article to a friend

BioMedNe

Research

Tools

Reviews

Journal

Collection

News &

Comment

Books &

Labware

Science

Jobs

Web

Links

Information for Advertisers

© Elsevier Science Limited 2000