Mouse anti-Human NHLRC1 Monoclonal Antibody | anti-NHLRC1 antibody

NHLRC1 (E3 Ubiquitin-protein Ligase NHLRC1, Malin, NHL Repeat-containing Protein 1, EPM2B, MGC119262, MGC119264, MGC119265) (FITC)

Cat. Num. AAA6148481

• Gene Names: NHLRC1; EPM2A; EPM2B; MALIN; bA204B7.2
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NHLRC1; Monoclonal Antibody; NHLRC1 (E3 Ubiquitin-protein Ligase NHLRC1; Malin; NHL Repeat-containing Protein 1; EPM2B; MGC119262; MGC119264; MGC119265) (FITC); anti-NHLRC1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 3G6
• Specificity: Recognizes human NHLRC1.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).

• Applicable Applications for anti-NHLRC1 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant protein corresponding to aa137-247 from human NHLRC1 (NP_940988) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: KTGRVVVVHDGRRRVKIFDSGGGCAHQFGEKGDAAQDIRYPVDVTITNDCHVVVTDAGDRSIKVFDFFGQIKLVIGGQFSLPWGVETTPQNGIVVTDAEAGSLHLLDVDF*
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (38.21kD).)

Related Product Information for anti-NHLRC1 antibody

E3 ubiquitin-protein ligase which in complex with EPM2A/laforin and HSP70 suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates PPP1R3C/PTG in a laforin-dependent manner, and targets it for proteasome-dependent degradation and this degradation decreases glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation.

Product Categories/Family for anti-NHLRC1 antibody

Antibodies; Ubiquitin Ligases

NCBI and Uniprot Product Information

• NCBI GI #: 40255283
• NCBI GeneID: 378884
• NCBI Accession #: NP_940988.2
• NCBI GenBank Nucleotide #: NM_198586
• UniProt Accession #: Q6VVB1; Q3SYB1; Q5VUK7; Q6IMH1
• Molecular Weight: 42,293 Da
• NCBI Official Full Name: E3 ubiquitin-protein ligase NHLRC1
• NCBI Official Synonym Full Names: NHL repeat containing E3 ubiquitin protein ligase 1
• NCBI Official Symbol: NHLRC1
• NCBI Official Synonym Symbols: EPM2A; EPM2B; MALIN; bA204B7.2
• NCBI Protein Information: E3 ubiquitin-protein ligase NHLRC1
• UniProt Protein Name: E3 ubiquitin-protein ligase NHLRC1
• Protein Family: E3 ubiquitin-protein ligase
• UniProt Gene Name: NHLRC1
• UniProt Synonym Gene Names: EPM2B

NCBI Description

The protein encoded by this gene is a single subunit E3 ubiquitin ligase. Laforin is polyubiquitinated by the encoded protein. Defects in this intronless gene lead to an accumulation of laforin and onset of Lafora disease, also known as progressive myoclonic epilepsy type 2 (EPM2).[provided by RefSeq, Mar 2010]

Uniprot Description

E3 ubiquitin-protein ligase. Together with the phosphatase EPM2A/laforin, appears to be involved in the clearance of toxic polyglucosan and protein aggregates via multiple pathways. In complex with EPM2A/laforin and HSP70, suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates the glycogen-targeting protein phosphatase subunits PPP1R3C/PTG and PPP1R3D in a laforin-dependent manner and targets them for proteasome-dependent degradation, thus decreasing glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation. Also promotes proteasome-independent protein degradation through the macroautophagy pathway.