Anti-Sacsin Antibody

About this product

Boster Bio Anti-Sacsin Antibody catalog # A00519. Tested in ELISA, IHC applications. This antibody reacts with Human, Mouse, Rat.

Specifications

Catalog number

A00519

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

Sacsin also known as DnaJ homolog subfamily C member 29 (DNAJC29) is a protein that in humans is encoded by the SACS gene. This gene consists of nine exons including a gigantic exon spanning more than 12.8k bp. It encodes the sacsin protein, which includes a UBQ region at the N-terminus, a HEPN domain at the C-terminus and a DnaJ region upstream of the HEPN domain. This modular protein is essential for normal mitochondrial network organization. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticityand peripheral neuropathy.

Synonyms

Sacsin; DnaJ homolog subfamily C member 29; DNAJC29; SACS; KIAA0730

Gene Name

SACS

Gene ID

26278

UniProt

Q9NZJ4

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E. coli-derived human Sacsin recombinant protein (Position: E3709-L3909) .

Clonality

Polyclonal

Tissue Specificity

Highly expressed in the central nervous system. Also found in skeletal muscle and at low levels in pancreas.

Applications

ELISA, IHC

Field of Research

Chaperones, Domain Families, Epigenetics and Nuclear Signaling, Hlh/Leucine Zipper, Metabolism, Mitochondrial Markers, Mitochondrial Metabolism, Pathways and Processes, Protein Trafficking, Signal Transduction, Transcription

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.

References & Citations

1. Entrez Gene: SACS spastic ataxia of Charlevoix-Saguenay (sacsin) . 2. Engert JC, Doré C, Mercier J, Ge B, Bétard C, Rioux JD, Owen C, Bérubé P, Devon K, Birren B, Melançon SB, Morgan K, Hudson TJ, Richter A (December 1999) . Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) : high-resolution physical and transcript map of the candidate region in chromosome region 13q11. Genomics. 62 (2) : 156–64. 3. Parfitt DA, Michael GJ, Vermeulen EG, Prodromou NV, Webb TR, Gallo JM, Cheetham ME, Nicoll WS, Blatch GL, Chapple JP (May 2009) . The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1. Human Molecular Genetics. 18 (9) : 1556–65

Storage Conditions

Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Specificity

No cross reactivity with other proteins.

Gene Name Synonym

Sacsin molecular chaperone

Subcellular Location

Cytoplasm.

Isotype

Rabbit IgG

Contents

Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.