WBSCR11 (K94) pAb

Williams-Beuren syndrome (WBS) is a developmental disorder caused by the hemizygous microdeletion on chromosome 7q11.23. WBS is an autosomal dominant genetic condition that is characterized by physical, cognitive and behavioral traits. The physical traits associated with WBS include facial dysmorphology, vascular stenoses, growth deficiencies, dental anomalies and neurologic and musculoskeletal abnormalities. Mild retardation, a weakness in visual-spatial skills, anxiety and a short attention span are typical cognitive and behavioral traits of WBS patients. The WBSCR11 gene is located within the WBS deletion and may contribute to the developmental symptoms found in WBS because of a loss of the encoded transcription factor. WBSCR11 is also designated GRF2IRD1, GTF3, Cream1 and MusTRD1 in human and BEN in mouse, due to slight differences in gene structure. WBSCR11 is expressed in all adult tissues as several variants and has discrete spatial and temporal expression during embryogenesis.

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SPECIFICATIONS

Catalog Number

BS2161

Size

100ug/100ul

Applications

WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

liquid

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Antigen

Synthetic peptide, corresponding to amino acids 63-112 of Human WBSCR11.

Species

WBSCR11 (K94) pAb detects endogenous levels of WBSCR11 protein.

Dilutions

WB: 1:500~1:1000

Swiss Prot

Q9UHL9

Storage Temp

Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.

Concentration

1ug/ul

Storage Buffer

1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.

Additional Info

For research use only, not for use in diagnostic procedure.

Molecular Weight

~ 106 kDa

Alternative Names

General transcription factor 3C polypeptide 3; TFIIIC102; Transcription factor IIIC 102 kDa subunit; TFIIIC-gamma; Transcription factor IIIC gamma subunit