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Concise Synthesis of the Unnatural Sphingosine and Psychosine Enantiomer.
[krabbe disease]
The
accumulation
of
psychosine
(
galactosyl
sphingosine
)
has
been
associated
with
the
pathogenesis
of
Krabbe
disease
,
however
,
the
exact
mechanism
of
its
cytotoxicity
remains
unclear
.
Herein
,
we
describe
the
synthesis
of
the
unnatural
enantiomer
of
erythrosphingosine
,
psychosine
,
and
related
derivatives
thereof
that
would
allow
for
the
mechanistic
elucidation
of
the
toxicity
of
psychosine
.
Diseases
Validation
Diseases presenting
"however"
symptom
adrenal incidentaloma
alexander disease
alpha-thalassemia
aromatase deficiency
benign recurrent intrahepatic cholestasis
cushing syndrome
dedifferentiated liposarcoma
dracunculiasis
dystrophic epidermolysis bullosa
esophageal carcinoma
focal myositis
heparin-induced thrombocytopenia
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
krabbe disease
lamellar ichthyosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
omenn syndrome
primary hyperoxaluria type 1
pyruvate dehydrogenase deficiency
sneddon syndrome
thoracic outlet syndrome
von hippel-lindau disease
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
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