Thermo Fisher Scientific Huntingtin Recombinant Rabbit Monoclonal Antibody (HL2483)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:3000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

HL2483

Immunogen

Recombinant fragment of human Huntingtin.

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Protein A

Storage buffer

PBS

Contains

no preservative

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Wet ice

RRID

AB_3093152

Target Information

Huntingtin is a disease gene linked to Huntingtons disease (HD), a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10-20 years.The huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. The genetic defect leading to Huntingtons disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.