You've been automatically redirected here from Humanzyme.com, which is now part of Proteintech Group. Click here to dismiss this alert.
Important Customer Notice Regarding Covid-19 Outbreak. Read more.

ANTIBODIES FOR NEURO-RELATED DISEASES: ALS AND FTD

Frontotemporal lobar degeneration (FTD) and amyotrophic lateral sclerosis (ALS): a short tale of two neurodegenerative diseases.

Frontotemporal lobar degeneration (FTD) and amyotrophic lateral sclerosis (ALS): a short tale of two neurodegenerative diseases.

Introduction

Neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), frontotemporal dementia (FTD), Huntington’s disease (HD), Parkinson’s disease (PD), spinocerebellar ataxia (SCA), and spinal muscular atrophies (SMAs), are incurable and debilitating disorders.

Amyotrophic Lateral Sclerosis (ALS)

ALS (also known as Lou Gehrig’s disease) is a common adult-onset neuropathological disease. Typical symptoms include hand and leg weakness, loss of ability to initiate/control movements, and slurred speech. This disorder is marked by the loss of motor neurons in the brain and spinal cord, and atrophy of the frontal and temporal lobes.

The majority of pathogenic mutations are present in TDP43 (also known as TAR DNA-binding protein 43 or TARDBP), fused in sarcoma (FUS; Figure 1), ubiquilin2 (UBQLN2; Figure 2), SOD1 (Figure 3), and also in the non-coding region of C9orf72.

FUS mono antibody validated in iHC

Figure 1. Immunohistochemical staining of paraffin-embedded human gliomas tissue slide using 60160-1-Ig (FUS/TLS monoclonal antibody) at a dilution of 1:1000 (under 10x lens). A heat mediated antigen retrieval step was performed with Tris-EDTA buffer (pH9).

UBQLN2 antibody validated in WB

Figure 2. Rat brain tissue lysate was subjected to SDS PAGE followed by western blot with 23449-1-AP (UBQLN2 antibody) at a dilution of 1:800 incubated at room temperature for 1.5 hours.


Related products for western blotting
Loading control antibodies
GAPDH Antibody
Catalog no.: 60004-1-Ig

GAPDH is commonly used as a protein loading control in western blot due to its consistently high expression in most cell types. This enzyme participates in several cellular events such as glycolysis, DNA repair, and apoptosis.

Proteintech monoclonal GAPDH antibodies are raised against a whole-protein antigen of human origin and have over 2,060 citations.

GAPDH antibody validated in immunofluorescent
Beta Actin Antibody (KD/KO validated)
Catalog no.: 66009-1-Ig 

Beta-actin is usually used as a loading control due to its broad and consistent expression across all eukaryotic cell types and the fact that expression levels of this protein are not affected by most experimental treatments.

66009-1-Ig has been cited in over 840 publications and has wide species reactivity.

beta actin antibody validated in immunofluorescent

Proteintech control antibodies are just $99 each (149 in Europe) for a 150ul size vial.

Ready-to-use protein ladders
Prestained Protein Ladder (free samples available) Broad Range Prestained Protein Ladder
Catalog no.: PL00001 Catalog no.: PL00002
Molecular weight coverage: 10-180 kDa Molecular weight coverage: 3-245 kDa
No. of markers: 10 prestained proteins No. of markers: 13 prestained proteins
Proteintech's new prestained protein marker (PL00001) is a three-color protein ladder with 10 markers covering a wide range of molecular weights from 10 - 180 kDa Proteintech's new broad range protein marker (PL00002) is a three-color protein standard with 13 markers covering a wide range of molecular weights from 3 - 245 kDa
Resolution of the prestained protein ladder in a 10-20% Tris-glycine gel (SDS-PAGE).  Resolution of the prestained protein ladder in a 4-12% Bis-Tris gel (SDS-PAGE)

SOD1 antibody validated in IHC

Figure 3. Immunohistochemical analysis of paraffin-embedded human testis using 10269-1-AP (SOD1 antibody) at dilution of 1:50 (under 10x lens). 

Frontotemporal Dementia (FTD)

FTD is a disease caused by progressive nerve cell loss. The majority of cases have brain atrophy in the frontal and/or temporal lobes. FTD is known as young-onset dementia and is characterized by behavioral abnormalities including a decline in cognitive, motor, and language function.

Most common FTD mutations are related to the TARDBP/TDP-43 protein (Figure 4), TAU (Figure 5) pathology, or the fused in sarcoma (FUS) gene.

C9ORF72 IN FTD AND ALS

ALS and FTD have been classified as belonging to the same family spectrum diseases, where hexanucleotide repeat expansions (HRE) of GGGGCC in C9orf72 is a key genetic cause player. Recent studies by Davidson Y et al. have confirmed the high specificity of Proteintech C9orf72 antibodies, highlighting their great value for research and diagnostic purposes.

TDP43 antibody validated in IHC

Figure 4. Immunohistochemistry of paraffin-embedded mouse brain tissue slide using 10782-2-AP (TDP43 antibody) at a dilution of 1:200 (under 10x lens).

TAU antibody validated in IF

Figure 5. Immunofluorescent analysis of (4% PFA) fixed mouse brain tissue using 10274-1-AP (TAU antibody) at a dilution of 1:50 and Alexa Fluor 488-conjugated AffiniPure Goat Anti-Rabbit IgG(H+L).

Related Antibodies

Product Type Applications Citations
TDP43 (C-Terminal) Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, chIP, ELISA 94
TDP43 (Human Specific) Antibody Mouse monoclonal WB, IP, IHC, FC, CoIP, ELISA 59
Recombinant TDP43 Antibody Rabbit recombinant, KD/KO validated WB, IHC, FC, ELISA -
FUS/TLS Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, chIP, ELISA 60
OPTN Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, ELISA 35
GFAP Antibody Mouse monoclonal WB, IP, IHC, ELISA 33
PTEN Antibody Mouse monoclonal WB, IHC, IF, ELISA 16
C9orf72 Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA 14
VAPB Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, ELISA 13
Ataxin 2 Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA 12
GR Repeat Antibody Rabbit polyclonal WB, IHC, IF, Dot blot, ELISA 12
GP Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA 10
PR Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA 9
P62/SQSTM1 Antibody Mouse monoclonal, KD/KO validated WB, IHC, IF, FC, ELISA 8
Granulin Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA 7
CHMP2B Antibody Rabbit polyclonal, KD/KO validated WB, IHC, IF, ELISA 6
Profilin 1 Antibody Rabbit polyclonal WB, IHC, IF, ELISA 6
VCP Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, CoIP, ELISA 5
GA Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA 5
AP Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA 4
TMEM106B Antibody Rabbit polyclonal WB, IP, IHC, IF, ELISA 3
Angiogenin Antibody Rabbit polyclonal WB, IHC, ELISA 1
P150 Glued Antibody Rabbit polyclonal WB, IHC, IF, ELISA 1
VGLUT1 Antibody Rabbit polyclonal IHC, IF, FC, ELISA 1
VCP Antibody Mouse monoclonal, KD/KO validated WB, IHC, IF, ELISA -
ALS2 Antibody Rabbit polyclonal WB, IP, IHC, IF, ELISA -
VGLUT2 Antibody Rabbit polyclonal WB, IHC, IF, ELISA -

Final remarks

New drugs should be developed to have neuroprotection properties via the improvement of synaptic plasticity, reduced oxidant and inflammation damage, or rescue mobility dysfunctions.

With an increasingly aging global population, the economic, as well as human, impact of neurodegenerative disorders is expected to increase unless reliable tools and care strategies can be developed. For example, recently, neuronal pentraxin 2 (NPTX2) has been identified as a reliable marker in f cerebrospinal fluid to monitor the progression of FTD. NPTX2 (10889-1-AP), also known as NARP (neuronal activity-regulated pentaxin), is secreted protein involved in excitatory synapse formation. It also plays a role in the clustering of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA)-type glutamate receptors at established synapses, resulting in non-apoptotic cell death of dopaminergic nerve cells.

 

Featured Products

Posted:
13 May, 2020

Share:


Back
to top