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2′,3′-Dihydrocephalomannine

$880

  • Brand : BIOFRON

  • Catalogue Number : BN-O1841

  • Specification : 98%(HPLC)

  • CAS number : 159001-25-9

  • Formula : C45H55NO14

  • Molecular Weight : 833.9

  • PUBCHEM ID : 146157441

  • Volume : 20mg

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Catalogue Number

BN-O1841

Analysis Method

Specification

98%(HPLC)

Storage

2-8°C

Molecular Weight

833.9

Appearance

Botanical Source

Structure Type

Category

SMILES

Synonyms

IUPAC Name

[(1S,4S,10S)-4,12-diacetyloxy-1,9-dihydroxy-15-[2-hydroxy-3-(2-methylbutanoylamino)-3-phenylpropanoyl]oxy-10,14,17,17-tetramethyl-11-oxo-6-oxatetracyclo[11.3.1.03,10.04,7]heptadec-13-en-2-yl] benzoate

Applications

Density

1.4±0.1 g/cm3

Solubility

Flash Point

511.8±34.3 °C

Boiling Point

922.6±65.0 °C at 760 mmHg

Melting Point

InChl

InChl Key

OKEKLOJNCOIPIT-FJLPVWCPSA-N

WGK Germany

RID/ADR

HS Code Reference

Personal Projective Equipment

Correct Usage

For Reference Standard and R&D, Not for Human Use Directly.

Meta Tag

provides coniferyl ferulate(CAS#:159001-25-9) MSDS, density, melting point, boiling point, structure, formula, molecular weight etc. Articles of coniferyl ferulate are included as well.>> amp version: coniferyl ferulate

No Technical Documents Available For This Product.

PMID

15832002

Abstract

Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is either in the emerin gene (X-linked recessive EDMD) or lamin A/C gene (autosomal dominant EDMD2 or LGMD1B). We report three cases of EDMD, EDMD2 and LGMD1B. A 14-yr-old boy showed limitation of cervical flexion and contractures of both elbows and ankles. Sinus arrest with junctional escape beats was noted. He was diagnosed as X-linked recessive EDMD (MIM 310300). A 28-yr-old female showed severe wasting and weakness of humeroperoneal muscles. Marked limitation of cervical flexion and contractures of both elbows and ankles were noted. Varying degrees of AV block were noted. She was diagnosed as autosomal dominant EDMD2 (MIM 181350). A 41-yr-old female had contractures of both ankles and limb-girdle type muscular dystrophy. ECG revealed atrial tachycardia with high grade AV block. She was diagnosed as autosomal dominant LGMD1B (MIM 159001). Cardiac dysrhythmias in EDMD and LGMD1B include AV block, bradycardia, atrial tachycardia, atrial fibrillation, and atrial standstill, causing sudden death necessitating pacemaker implantation. Cardiologists should know about these unusual genetic diseases with conduction defects, especially in young adults.

KEYWORDS

Muscular Dystrophies, Cardiomyopathies, emerin, Lamins, Heart Conduction System

Title

Cardiac Dysrhythmias, Cardiomyopathy and Muscular Dystrophy in Patients with Emery-Dreifuss Muscular Dystrophy and Limb-Girdle Muscular Dystrophy Type 1B

Author

Jong-Seo Hong,*¶ Chang-Seok Ki,† Jong-Won Kim,† Yeon-Lim Suh,‡ June Soo Kim,* Kyung Kee Baek,* Byoung Joon Kim,§ Kyoung Ju Ahn,∥ and Duk-Kyung Kimcorresponding author*

Publish date

2005 Apr;

PMID

29770364

Abstract

Three cases of delated cardiomyopathy (DCM) with conduction defects (OMIM 115200), limb girdle muscular dystrophy 1B (OMIM 159001) and autosomal dominant Emery-Dreifuss muscular dystrophy 2 (OMIM 181350), all associated with different LMNA mutations are presented. Three heterozygous missense mutations were identified in unrelated patients – p.W520R (c.1558T > C), p.T528R (с.1583С > G) and p.R190P (c.569G > C). We consider these variants as pathogenic, leading to isolated DCM with conduction defects or syndromic DCM forms with limb-girdle muscular dystrophy and Emery-Dreifuss muscular dystrophy. The mutations were not detected in the ethnically matched control group and publicly available population databases. Their de novo occurrence led to the development of the disease that was not previously detected in the extended families. Mutations at the same codons associated with laminopathies have been already reported. Differences in the clinical phenotype for p.R190P and p.T528R carrier patients are shown and compared to previous reports.

KEYWORDS

dilated cardiomyopathy, limb-girdle muscular dystrophy, Emery-Dreifuss muscular dystrophy

Title

Three new cases of dilated cardiomyopathy caused by mutations in LMNA gene

Author

LARYSA N. SIVITSKAYA,1 NINA G. DANILENKO,1 TATIYANA G. VAIKHANSKAYA,2 TATSIYANA V. KURUSHKA,2 and OLEG G. DAVYDENKO1

Publish date

2017 Dec;

PMID

14569138

Title

A synonymous codon change in the LMNA gene alters mRNA splicing and causes limb girdle muscular dystrophy type 1B

Author

A Todorova, B Halliger-Keller, M Walter, M Dabauvalle, H Lochmuller, and C Muller

Publish date

2003 Oct