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Sly syndrome

Mucopolysaccharidosis Type VII or Sly syndrome (named after its discoverer William Sly in 1969) is also sometimes called MPS. The defective gene lies on chromosome 7. MPS is transmitted as an autosomal recessive trait. more...

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It is an extremely rare inherited metabolic disorder characterized by a deficiency of the enzyme β-glucuronidase, a lysosomal enzyme. Sly syndrome belongs to a group of disorders known as the mucopolysaccharidoses, which are lysosomal storage diseases. In Sly syndrome, the deficiency in β-glucuronidase leads to the accumulation of certain complex carbohydrates (mucopolysaccharides) in many tissues and organs of the body.

The symptoms of Sly syndrome are similar to those of Hurler syndrome (MPS I). The symptoms include:

  • in the head, neck, and face: coarse (Hurler-like) facies and macrocephaly, frontal prominence, premature closure of sagittal lambdoid sutures, and J-shaped sella turcica
  • in the eyes: corneal opacity and iris colobmata
  • in the nose: anteverted nostrils and a depressed nostril bridge
  • in the mouth and oral areas: prominent alveolar processes and cleft palate
  • in the thorax: usually pectus carinatum or exacavatum and oar-shaped ribs; also a protruding abdomen and inguinal or umbilical hernia
  • in the extremities: talipes, an underdeveloped ilium, aseptic necrosis of femoral head, and shortness of tubular bones occurs
  • in the spine: kyphosis or scoliosis and hook-like deformities in thoracic and lumbar vertebrate
  • in the bones: dysotosis multiplex

In addition recurrent pulmonary infections occur. Hepatomegaly occurs in the gastrointestinal system. Splenomegaly occurs in the hematopoietic system. Inborn mucopolysaccharide metabolic disorders due to β-glucuronidase deficiency with granular inclusions in granulocytes occurs in the biochemical and metabolic systems. Growth and motor skills are affected, and mental retardation also occurs.

MPS type VII occurs in only 1:250,000 people.

Mucopolysaccahridosis Type VII is also known as β-glucurondinase deficiency, β-glucurondinase deficiency mucopolysaccahridosis, GUSB deficiency, mucopolysaccahride storage disease VII, MCA, and MR.

Read more at Wikipedia.org


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Freedom Rock
From Washingtonpost.com, 12/7/05

Byline: J. Freedom du Lac

Washington Post music critic J. Freedom du Lac is online every Wednesday at 2 p.m. ET to talk about the latest on the music scene: hip-hop, pop, alternative, country, alt-country, rock, reggae, reggaeton, R&B and whatever it is that Ashlee Simpson does.

The transcript follows.

____________________

J. Freedom du Lac: Hola, musichounds. My ears are still ringing from last night's Sinead O'Connor show at the 9:30. Not from the music itself (which was most righteous), but from the obnoxious woman who camped out at the bar upstairs.

The woman would. Not. SHUT. UP. She gabbed away through a good chunk of the show and didn't seem to pay any attention to the music. (That I never once saw her looking at the stage was a pretty good indicator.) Unfortunately, she had one of those piercing voices that would not/could not be denied, like a dog whistle for humans. She was whistling a most asinine tune, too: "So my friend, who's a model, blahblahblah, etc, blah, etc."

Can anybody out there in chatland explain why a person would pay $40 to attend a concert s/he clearly doesn't care about, just to hang out at the bar and talk all night? Not the most ideal chatting conditions. And, of course, it's just flat-out rude. Were Stoney's, the Red Room and the Saint-Ex closed last night or something? Good lord.

Onward.

_______________________

Carmel, IN: The High Dials' disc hasn't arrived in the mail yet, so your streak remains intact for another week. In the meantime, how about sharing a list of 10 records you're taking with you to that proverbial desert island.

J. Freedom du Lac: That's because the mail carrier is a Freedom Rock reader and poached the disc. Ooops!

I've been working on my 2005 Top 10 (+10) list. That'll run at the end of the year, at which point I'd love to hear everybody else's '05 Top 10. And then we can move on to D.I.D. lists.

_______________________

Charlotte, NC: What music movie biography did you like better - "Walk the Line" or "Ray"?

J. Freedom du Lac: "Ray." Though both movies were flawed.

_______________________

Loud Fans: I often wonder this myself.... though I have heard of people not wanting to go to a concert alone so they drag a friend along that doesn't really have any interest but figured they'd go along. Or even that a parent is there for a kid and thus the parent just hangs out at the bar...

Whatever the reason it drives me nuts! Especially when they don't shut up when they are sitting right next to you, or when they go overboard on the drinks and get up to use the restroom every 10 mins...arrrgh

J. Freedom du Lac: Ah, yes - the small-bladder syndrome. There was a couple sitting on the stools in front of me last night, and I think one of them got up at least once every two songs. Good times!

_______________________

Washington, DC: Peak-era Funkadelic vs. peak-era Sly Stone. Who wins?

J. Freedom du Lac: Sly by 10 yards. Funny you'd ask - I'm working on a Sly Stone story, and I talked to George Clinton about the guy. George bows down at Sly's feet. He tells a pretty hilarious story about the first time he heard Sly's stuff. You'll giggle when you read it, I think.

_______________________

Arlington, VA: Did you see Sinead last night? I love her, and I dig the reggae. Her voice was a little raspy, though.

J. Freedom du Lac: I'm writing the review as we speak. Actually, I'm not writing it as we speak because, we'll, we're speaking. But yeah, I liked it for the most part, though she wasn't in great voice (she's out of training, having been on the beach for two years). And I thought she did a little bit too much of that I'm-gonna-wail-while-the-mic-is-at-my-hip thing. She has an incredible voice; it'd be nice to hear it a little more.

_______________________

Baltimore, MD: Did you catch the Eminem concert on Showtime? Was it me or was the show kind of ... I don't know, wack? It may be unfair to criticize a televised concert, but I've seen other concerts on that same network (No Doubt, Usher) and they were way better.

J. Freedom du Lac: I did not see it. I believe they taped it shortly after the Anger Management show at Nissan, so I wasn't expecting to anything particularly new or groundbreaking. Though I was kind of curious to see how his zit looked in hi-def. When Em played here, that thing looked like Mount Vesuvius.

_______________________

free whorled : bro. rock, you might actually have good taste. i'm a 49er, fading fast... can you hip me to some current noise to keepeth me up to date and masquerading young? I liked andrew bird, which was referenced via here, and mike ness (some) and mike henderson (great scott, not the country crap). I've got High Dials waiting. Juliana Hatfield? Neko Case? thanks, lad.

J. Freedom du Lac: Today's tip: The group is World Leader Pretend, the album is "Punches," and it's pretty tasty stuff. Might be too countryish for you, but I'm also liking the Sons and Daughters album, "The Repulsion Box." It ain't pure country by any stretch, though.

_______________________

Loudmouths with comps: Obviously, she knew somebody, or works somewhere, and got the ticket free.

I had to shame some people at a Lyle Lovett amphitheater show a few years ago when they sat down behind us and started MAKING phone calls! I told them that we had all spent the same $60 for our tickets and would they please shut up...the guys in their group all subtly nodded their heads and the Chatty Cathies all gave us this nasty look.

Then they got up and moved to better seats, I suppose to annoy people with more money.

J. Freedom du Lac: Maybe her friend the model knew somebody who knew somebody.

Pretty funny about the folks at the Lyle show. Glad to hear that you didn't have to suffer through the entire show with the blabbermouths working their magic behind you.

_______________________

Olney, MD. : Listen, it's not EASY being a music lover and having a small bladder.

Great Marketing Opportunity - In a few years the Rolling Stones sign up Depends a tour sponsor: "Don't leave your seat, mates, rock on with Depends - Mick does!"

J. Freedom du Lac: Ouch! Though I've gotta tell you: Mick was wearing some really tight pants at the MCI Center, and my seats were very, very close to the stage. If he'd been wearing Depends, I think I would've noticed.

_______________________

Frederick, MD: Any chance of Snoop Dogg doing a Christmas CD?

J. Freedom du Lac: Come on, now - you mean to tell me that you don't own a copy of "Christmas on Death Row"? Snoop does "Santa Goes Straight to the Ghetto." It's not James Brown, but still.

_______________________

Montgomery County: What advice would you give to a local band that is trying to get their self-released CD reviewed by local critics? Do you prefer to write about artists on labels? I've been told the best way to get noticed and reviewed is to be a band that plays out often, but it's also hard to get gigs if your press kit is short on quotes/reviews, etc. The old Catch-22 ... Give me hope!!

J. Freedom du Lac: Send me your stuff and I'll give it a spin. No promises, but it certainly won't hurt.

1150 - 15th St. NW

Washington, DC 20071

_______________________

Alexandria, VA: How long did George Clinton have to wait before he heard Sly for the first time ? Please answer in atomic dog years.

J. Freedom du Lac: This sounds like a riddle wrapped in a mystery inside a Zig Zag.

_______________________

Washington, DC: Last week you said that Robert Hilburn was taking a buyout from the L.A. Times. This week I saw on the Boston Globe's Web site that Steve Morse is retiring. Those are two big losses to take. I always enjoyed reading both of them.

J. Freedom du Lac: Strange things are afoot in the newspaper biz. Alas.

I got a kick out of reading today that Bono showed up at Steve Morse's retirement party. He said some very nice things about the guy. As he should have, since Morse gave that band a lot of love from Day One.

Wonder if Springsteen will show for Hilburn's party.

_______________________

Capitol Hill, Washington, DC: I am embarrassed to say that I have never been to a concert in DC that wasn't at the 9:30 club. Are there any good shows that will be at the Black Cat, DC9, Iota, or any of the other places in the next few months? The last couple of CDs I have purchased have been the new Franz Ferdinand and Maximo Park if that helps.

J. Freedom du Lac: Hmmm. At the Black Cat, try Trans Am on Dec. 29. I think they might be just your speed. If you're not booked up tonight, you might investigate Akron/Family at Iota, though only if you have a psychedelic itch that needs to be scratched.

By the way, have you ever looked up into the rafters at the 9:30? Check it out the next time you're there, especially if the house lights are on. The dust bunnies have taken over.

_______________________

Tenleytown, Washington, DC: J. Freelove: The Business section today reported that the upcoming Bon Jovi show at MCI Center is sold out except for a few singles and obstructed view seats. For the love of everything righteous about rock n roll, how can this be possible? They are a thoroughly mediocre band, with nothing memorable in their repertoire, and well past their expiration date. How is this possible?

J. Freedom du Lac: Choose one:

(a) There's no accounting for taste.

(b) There is no justice.

(c) The Heather Locklear effect.

(d) The Triumph the Insult Comic Dog effect.

_______________________

Falls Church, VA: Hey, get this -- I went to a Sly Stone concert once and he actually showed up !!

J. Freedom du Lac: Sure, but was he four hours late? DC was a particularly tough town for that band.

_______________________

Dear God: Just a note: iTunes finally added the XTC catalog to their library!

J. Freedom du Lac: (Insert "Andy Partridge in a pear tree" joke here.)

_______________________

Arlington, VA: I have an iTunes question. Is it iTunes fault that they don't offer beatles, zeppelin, ac/c, etc.? Or is it that artist? Will these artists be added anytime in the future? How does this all work?? Isn't it to the artist's advantage to have their songs available on iTunes?? How about Seger & Silver Bullet Band? I'm sure I could run a six minute mile if I had Ramblin Gamblin Man on my mix.

J. Freedom du Lac: That's on the artists - or at least the folks who control the master recordings. The Beatles are the biggest of the holdouts. You can be sure that iTunes, Rhapsody, Yahoo Music, et al, would love to have that catalogue available. I believe the Stones' stuff only recently surfaced in the legal online arena.

Course, you don't need to wait for the stuff to show up on iTunes to put it on your iPod. You can always rip the CD itself. There are, ahem, other ways to get music online, too, but I would not advise doing so without consulting your attorney. But hey, if you're really trying to become the next Frank Shorter...

_______________________

Arlington, VA: Any word on how much "Holiday" music one might expect at the Roches reunion show at the Birchmere?

J. Freedom du Lac: I've got nothing. Anyone?

_______________________

Omaha, NE: Mr. du Lac:

First off, I must say you are in possession of the coolest name for a music critic. Second, I am curious about your comments regarding Conor Oberst and his various musical functionaries and offerings. I quite like him. He is combination of the poet and rebel wrapped around a romantic freak that appeals to me. As someone new to your chat, I have not been exposed to your opinions, if, in fact, you have seen or heard him.

Also, my new fav CD is Death Cab for Cutie's "Plans". Comments? I like it but it's not exactly Chuck Berry, now is it, love?

thanks.

J. Freedom du Lac: I love me some Bright Eyes. Producer David is gonna make life easy for you and post a link.

I am not on the Death Cab bandwagon, though. I don't hate the album by any stretch, but I don't love it, either. And I wasn't wowed by them live, either - though the light show was pretty cool. Not so cool: The fact that I felt like I was at a meet-and-greet with the cast of "The O.C."

_______________________

Jovi: "They are a thoroughly mediocre band, with nothing memorable in their repertoire"

Come on now. They may not be the best band but they definitely have a few memorable songs.

Livin' On a Prayer

You Give Love a Bad Name

J. Freedom du Lac: True, but memorable doesn't necessarily equal good. After all, I remember quite a few Michael Bolton songs.

But yeah, Bon Jovi isn't terrible. I'll take them over Journey any day.

_______________________

washingtonpost.com: Bright Eyes, Over Two Dark Circles

_______________________

Dupont Circle, Washington, DC: Snoop's done a few contributions to Christmas compilations. My fav is "Christmastime in the LBC," which contains the poignant lyric "I'll stick my yuletide log in your [somehwhere it doesn't belong]/Then dress you up nice take you to midnight Mass"." Ahhh, the holidays!

J. Freedom du Lac: Ah, yes - a Christmas classic! I can see Snoop remaking "Chestnuts Roasting On An Open Fire," too. Though "deez" would have to factor prominently into the new lyric.

_______________________

Speaking of Sly: Is there any group currently famous for their unreliability when it comes to live shows ?

Can any artist in history match Sly's performance in this arena ? George Jones maybe ?

J. Freedom du Lac: No-Show Jones! Good call.

Guns N Roses didn't exactly put promoters at ease.

But I don't think anybody can top Sly's track record. I think Sly and the Family Stone gave more promoters more ulcers than any act in history.

_______________________

Durham, NC: I own only one (1) Christmas CD (Booker T. and the MGs). Is that enough, or would it behoove me to buy more?

J. Freedom du Lac: James Brown's Funky Christmas. It's great. (Seriously.)

_______________________

Springfield, VA: Is it OK to go to a concert by yourself? I never get asked to go and don't want to go alone. In the past 22 years, I've been to 7 concerts and I feel like I've missed out on something by refusing to go alone.

J. Freedom du Lac: Absolutely. I do it all the time, when friends bail out at the last minute or nobody wants to go in the first place, because the artist doesn't interest anybody in my plus-one rotation. (Certainly, it's not because of the company. I mean, who WOULDN'T want to hang out with me?)

I do think it's easier/less awkward to fly solo at a GA show than at a concert where there's reserved seating.

Seven shows in 22 years, eh? That's like a two-week stretch for me these days!

_______________________

Sterling, VA: I listened to Yankee Hotel Foxtrot for the first time a few weeks ago, and have been absolutely stunned at how good this band is.

Why have I never heard of Wilco until recently?

J. Freedom du Lac: Because you've missed all the books and DVDs and extensive critical drooling? Wilco has a rather remarkable critical praise to album sales ratio. It's off the charts, really.

_______________________

Louisville, KY: Did you happen to see Dave Matthews Band on PBS last week? I hope the performance of "Louisiana Bayou" with Robert Randolph and Rashawn Randolph helped get you over your anti-DMB stance.

Also, as someone who trades bootleg recordings, obnoxious, talking fans are the bane of my existence. I've got a recording of some guy hitting on a girl all throughout a Rufus Wainwright show. What's the point?

J. Freedom du Lac: I did not happen to see DMB on PBS. So, I've yet to be converted.

Did the guy at the Rufus Wainwright show wind up getting the girl? Or was it all for naught?

_______________________

Washington, D.C.: Looking for smallish shows? You should check out the band Voxtrot at the Warehouse Next Door on Saturday. At $7, its a no-brainer.

They will be playing much bigger venues in future trips to DC.

J. Freedom du Lac: The People Have Spoken.

_______________________

DM fan in Arlington: Hey J.F.duL.! I have tix to go see Depeche Mode Friday night at the Patriot Center and I'm very excited - last time I saw them was on the Violator tour, which was great. Have you heard anything about this tour so far? Dave Gahan sounds great on the new album and I'm hoping they sound as good live.

Thanks - and love your work!

J. Freedom du Lac: A good friend of mine saw them in California and loved the show. Declared it the best concert he's been to this year. And he's been to a bunch. Everything an arena show should be, was his executive summary.

_______________________

Washington, DC: Just wanted to say that Monday at 9:30, I saw John Doe of X and Juliana Hatfield sing a duet of "When Will I be Loved." It was friggin' spectacular.

J. Freedom du Lac: I wish I'd gone to that show. I've heard nothing but good things.

_______________________

Omaha, NE: Mr. du Lac:

If you had to rescue five albums (my god, am I dating myself) from a fire at your home, knowing they would never be heard again unless you saved them, what would they be?

Thanks.

J. Freedom du Lac: Well, the first thing I'd grab is my coveted copy of "Y Kant Tori Read," by Tori Amos. That thing is worth some serious cash. In fact, when I was interviewing Tori many moons ago, I mentioned that I owned a copy of the album, and she highly encouraged me to sell it. Because, (a) it's terrible. And (b), she said: "You can use the money to buy a great bottle of Champagne."

I have some live boots that would probably fill the 2-5 slots. Tom Petty, Gillian Welch, etc. Or, maybe I'd grab my 7-inch of Rage Against the Machine covering NWA's "F--- Tha Police." Hell, I dunno. I'm pretty sure I'd rescue more than five albums. I have a pretty big suitcase that could hold several hundred CDs. Plus, of course, my live Loggins and Messina DVD.

_______________________

California: Happy Pearl Harbor Day. Any recordings that recently caught you unaware with out unexpectedly good they were?

J. Freedom du Lac: I didn't know today was a celebratory kinda of holiday. Hmmm. The last time I was at Pearl Harbor was Sept. 10, 2001. For reals.

As for surprisingly good albums: The High Dials' "War of the Wakening Phantoms" and Erin McKewon's "We Will Become Like Birds" both jumped from my CD bin into heavy rotation in the last week.

_______________________

Arlington, VA: I have a penchant for 80's New Wave/New Romantics music. Is there anything wrong with me?

J. Freedom du Lac: Yes, absolutely. For one thing, you need to get rid of your parachute pants. They're not flattering anymore.

Thanks for stopping by this week, folks. Sinead O'Connor review runs tomorrow. Maybe the blabbermouth will read it to see what she missed. (But probably not. Somehow, I suspect the only thing she's reading these days is Lucky. and maybe Cosmo.)

_______________________

Y Kant I have that CD: Whoa! Feeling up for a trade for that Y Kant Tori Read CD? hehe

Not all the songs are terrible. In fact she plays 2 of them fairly regularly, Cool On Your Island and Etienne. Did you happen to check out her bootleg box set that came out this week of 6 different shows?

J. Freedom du Lac: If you have a bottle of 1988 Krug Clos du Mesnil, I'm all ears.

_______________________

Sterling, VA: J. Freedom du Lac: Because you've missed all the books and DVDs and extensive critical drooling? Wilco has a rather remarkable critical praise to album sales ratio. It's off the charts, really.

I must be living under a rock then to never have heard Wilco before, and I feel like I've been missing out!

I really think they're the best band I've heard in about ten years, if not longer. Stunningly, amazingly talented.

J. Freedom du Lac: OK, I'm really going now. But first, a brief bit of heresy: I like Son Volt (at least the early stuff) more than I like Wilco.

Which, if you're a Wilco newcomer, is a significant vis-a-vis because Wilco's Jeff Tweedy was in the late, great alt-country band Uncle Tupelo with Son Volt's Jay Farrar. And if you haven't heard Uncle Tupelo, you really need to do yourself a favor and investigate them.

_______________________

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