Name: SACS Antibody (PACO54202)
SKU: PACO54202
Availability: OutOfStock

Antibody Name:	SACS Antibody (PACO54202)
Antibody SKU:	PACO54202
Size:	50ug
Host Species:	Rabbit
Tested Applications:	ELISA, IHC, IF
Recommended Dilutions:	ELISA:1:2000-1:10000, IHC:1:20-1:200, IF:1:200-1:500
Species Reactivity:	Human
Immunogen:	Recombinant Human Sacsin protein (983-1150AA)

Form:	Liquid
Storage Buffer:	Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Purification Method:	>95%, Protein G purified
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Non-conjugated

	Immunohistochemistry of paraffin-embedded human brain tissue using PACO54202 at dilution of 1:100.
	Immunofluorescence staining of A549 cells with PACO54202 at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
	Immunohistochemistry of paraffin-embedded human placenta tissue using PACO54202 at dilution of 1:100.

Background:	Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.
Synonyms:	Sacsin (DnaJ homolog subfamily C member 29) (DNAJC29), SACS, KIAA0730

UniProt Protein Function:
UniProt Protein Details:
NCBI Summary:	This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al., PubMed: 19208651). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, May 2013]
UniProt Code:	Q9NZJ4
NCBI GenInfo Identifier:
NCBI Gene ID:	26278
NCBI Accession:
UniProt Secondary Accession:	Q9NZJ4
UniProt Related Accession:	Q9NZJ4
Molecular Weight:	Observed MW: 521kDa
NCBI Full Name:
NCBI Synonym Full Names:	sacsin molecular chaperone
NCBI Official Symbol:	SACS
NCBI Official Synonym Symbols:	SPAX6; ARSACS; DNAJC29; PPP1R138
NCBI Protein Information:	sacsin
UniProt Protein Name:
UniProt Synonym Protein Names:
Protein Family:	Sacsin
UniProt Gene Name:
UniProt Entry Name: