357 research outputs found
Mevalonate kinase deficiencies: from mevalonic aciduria to hyperimmunoglobulinemia D syndrome
Mevalonic aciduria (MVA) and hyperimmunoglobulinemia D syndrome (HIDS) represent the two ends of a clinical spectrum of disease caused by deficiency of mevalonate kinase (MVK), the first committed enzyme of cholesterol biosynthesis. At least 30 patients with MVA and 180 patients with HIDS have been reported worldwide. MVA is characterized by psychomotor retardation, failure to thrive, progressive cerebellar ataxia, dysmorphic features, progressive visual impairment and recurrent febrile crises. The febrile episodes are commonly accompanied by hepatosplenomegaly, lymphadenopathy, abdominal symptoms, arthralgia and skin rashes. Life expectancy is often compromised. In HIDS, only febrile attacks are present, but a subgroup of patients may also develop neurological abnormalities of varying degree such as mental retardation, ataxia, ocular symptoms and epilepsy. A reduced activity of MVK and pathogenic mutations in the MVK gene have been demonstrated as the common genetic basis in both disorders. In MVA, the diagnosis is established by detection of highly elevated levels of mevalonic acid excreted in urine. Increased levels of immunoglobulin D (IgD) and, in most patients of immunoglobulin A (IgA), in combination with enhanced excretion of mevalonic acid provide strong evidence for HIDS. The diagnosis is confirmed by low activity of mevalonate kinase or by demonstration of disease-causing mutations. Genetic counseling should be offered to families at risk. There is no established successful treatment for MVA. Simvastatin, an inhibitor of HMG-CoA reductase, and anakinra have been shown to have beneficial effect in HIDS
Measurement of Exclusive B Decays to Final States Containing a Charmed Baryon
Using data collected by the CLEO detector in the Upsilon(4S) region, we
report new measurements of the exclusive decays of B mesons into final states
of the type Lambda_c^+ p-bar n(pi), where n=0,1,2,3. We find signals in modes
with one, two and three pions and an upper limit for the two body decay
Lambda_c^+ pbar. We also make the first measurements of exclusive decays of B
mesons to Sigma_c p-bar n(pi), where n=0,1,2. We find signals in modes with one
and two pions and an upper limit for the two body decay Sigma_c p-bar.
Measurements of these modes shed light on the mechanisms involved in B decays
to baryons.Comment: 11 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLNS, submitted to PR
Measurement of the Masses and Widths of the Sigma_c^++ and Sigma_c^0 Charmed Baryons
Using data recorded by the CLEO II and CLEO II.V detector configurations at
CESR, we report new measurements of the masses of the Sigma_c^{++} and
Sigma_c^0 charmed baryons, and the first measurements of their intrinsic
widths. We find M(Sigma_c^{++}) - M(Lambda_c^+) = 167.4 +- 0.1 +- 0.2 MeV,
Gamma(Sigma_c^{++}) = 2.3 +- 0.2 +- 0.3 MeV, and M(Sigma_c^0) - M(Lambda_c^+) =
167.2 +- 0.1 +- 0.2 MeV, Gamma(Sigma_c^0) = 2.5 +- 0.2 +- 0.3 MeV, where the
uncertainties are statistical and systematic, respectively.Comment: 9 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLNS, submitted to PRD, Rapid
Communications. Reference [13] correcte
Evidence for the Decay
We present a search for the ``wrong-sign'' decay D0 -> K+ pi- pi+ pi- using 9
fb-1 of e+e- collisions on and just below the Upsilon(4S) resonance. This decay
can occur either through a doubly Cabibbo-suppressed process or through mixing
to a D0bar followed by a Cabibbo-favored process. Our result for the
time-integrated wrong-sign rate relative to the decay D0 -> K- pi+ pi- pi+ is
(0.0041 +0.0012-0.0011(stat.) +-0.0004(syst.))x(1.07 +-0.10)(phase space),
which has a statistical significance of 3.9 standard deviations.Comment: 9 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLNS, submitted to PR
Hadronic Mass Moments in Inclusive Semileptonic B Meson Decays
We have measured the first and second moments of the hadronic mass-squared
distribution in B -> X_c l nu, for P(lepton) > 1.5 GeV/c. We find <M_X^2 -
M_D[Bar]^2> = 0.251 +- 0.066 GeV^2, )^2 > = 0.576 +- 0.170
GeV^4, where M_D[Bar] is the spin-averaged D meson mass.
From that first moment and the first moment of the photon energy spectrum in
b -> s gamma, we find the HQET parameter lambda_1 (MS[Bar], to order 1/M^3 and
beta_0 alpha_s^2) to be -0.24 +- 0.11 GeV^2. Using these first moments and the
B semileptonic width, and assuming parton-hadron duality, we obtain |V_cb| =
0.0404 +- 0.0013.Comment: 11 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLNS, submitted to PR
Observation of Exclusive barB --> D(*) K*- Decays
We report the first observation of the exclusive decays \bar B\to
D^{(*)}K^{*-}, using 9.66 x 10^{6} B\bar{B} pairs collected at the \Upsilon(4S)
with the CLEO detector. We measure the following branching fractions: {\cal
B}(B^- -> D^0 K^{*-})=(6.1 +- 1.6 +-1.7)x10^{-4}, {\cal B}(\bar{B^0} ->
D^+K^{*-})=(3.7 +- 1.5 +- 1.0) x 10^{-4}, {\cal B}(\bar{B^0} ->
D^{*+}K^{*-})=(3.8 +- 1.3 +- 0.8) x 10^{-4} and {\cal B}(B^- --> D^{*0}
K^{*-})=(7.7 +- 2.2 +- 2.6) x 10^{-4}. The \bar B ->D^*K^{*-} branching ratios
are the averages of those corresponding to the 00 and 11 helicity states. The
errors shown are statistical and systematic, respectively.Comment: 9 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLNS, Published in
Phys.Rev.Lett.88:101803,200
Observation of the Charmed Baryon at CLEO
The CLEO experiment at the CESR collider has used 13.7 fb of data to
search for the production of the (css-ground state) in
collisions at {\rm GeV}. The modes used to
study the are ,
, , , and
. We observe a signal of 40.49.0(stat) events
at a mass of 2694.62.6(stat)1.9(syst) {\rm MeV/}, for all modes
combined.Comment: 10 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLN
Observation of and
We have studied two-body charmless hadronic decays of mesons into the
final states phi K and phi K^*. Using 9.7 million pairs collected
with the CLEO II detector, we observe the decays B- -> phi K- and B0 -> phi K*0
with the following branching fractions: BR(B- -> phi K-)=(5.5 +2.1-1.8 +- 0.6)
x 10^{-6} and BR(B0 -> phi K*0)=(11.5 +4.5-3.7 +1.8-1.7) x 10^{-6}. We also see
evidence for the decays B0 -> phi K0 and B- -> phi K*-. However, since the
statistical significance is not overwhelming for these modes we determine upper
limits of <12.3 x 10^{-6} and <22.5 x 10^{-6} (90% C.L.) respectively.Comment: 9 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLN
Evidence of New States Decaying into
Using 13.7 of data recorded by the CLEO detector at CESR, we report
evidence for two new charmed baryons: one decaying into
with the subsequent decay , and its
isospin partner decaying into followed by
. We measure the following mass differences
for the two states: =318.2+-1.3+-2.9 MeV,
and =324.0+-1.3+-3.0 MeV. We interpret
these new states as the particles, the charmed-strange
analogs of the .Comment: 10 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLN
Measurement of the Relative Branching Fraction of to Charged and Neutral B-Meson Pairs
We analyze 9.7 x 10^6 B\bar{B}$ pairs recorded with the CLEO detector to
determine the production ratio of charged to neutral B-meson pairs produced at
the Y(4S) resonance. We measure the rates for B^0 -> J/psi K^{(*)0} and B^+ ->
J/psi K^{(*)+} decays and use the world-average B-meson lifetime ratio to
extract the relative widths f+-/f00 = Gamma(Y(4S) -> B+B-)/Gamma(Y(4S) ->
B0\bar{B0}) = = 1.04 +/- 0.07(stat) +/- 0.04(syst). With the assumption that
f+- + f00 = 1, we obtain f00 = 0.49 +/- 0.02(stat) +/- 0.01(syst) and f+- =
0.51 +/- 0.02(stat) +/- 0.01(syst). This production ratio and its uncertainty
apply to all exclusive B-meson branching fractions measured at the Y(4S)
resonance.Comment: 11 pages postscript, also available through
http://w4.lns.cornell.edu/public/CLN
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