Peter munk and erwin schaeffer
Abstract
Amyotrophic lateral sclerosis is a fatal multisystemic neurodegenerative disease with motor neurons being a primary target. Although progressive weakness is a hallmark feature of amyotrophic lateral sclerosis, there is considerable heterogeneity, including clinical presentation, progression, and the underlying triggers for disease initiation. Based on longitudinal studies with families harboring amyotrophic lateral sclerosis-associated gene mutations, it has become apparent that overt disease is preceded by a prodromal phase, possibly in years, where compensatory mechanisms delay symptom onset. Since 85–90% of amyotrophic lateral sclerosis is sporadic, there is a strong need for identifying biomarkers that can detect this prodromal phase as motor neurons have limited capacity for regeneration. Current Food and Drug Administration-approved therapies work by slowing the degenerative process and are most effective early in the disease. Skeletal muscle, including the neuromuscular junction, manifests abnormalities at the earliest stages of the disease, before motor neuron loss, making it a promising source for identifying biomarkers of the prodromal phase. The accessibility of muscle through biopsy provides a lens into the distal motor system at earlier stages and in real time. The advent of “omics” technology has led to the identification of numerous dysregulated molecules in amyotrophic lateral sclerosis muscle, ranging from coding and non-coding RNAs to proteins and metabolites. This technology has opened the door for identifying biomarkers of disease activity and providing insight into disease mechanisms. A major challenge is correlating the myriad of dysregulated molecules with clinical or histological progression and understanding their relevance to presymptomatic phases of disease. There are two major goals of this review. The first is to summarize some of the biomarkers identified in human amyotrophic lateral sclerosis muscle that have a clinicopath
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Overview
- Interview Summary
- Irving Schaffer, born May 26, 1928 in Chust, Czechoslovakia (present day Khust, Ukraine), discusses growing up in an Orthodox family; his early education; a teacher beating him in front of the class in 1943; experiencing antisemitism before the war; his father’s arrest for receiving milk from a neighbor; the deportation of his father and brother to labor camps; his family’s eviction from their home and deportation to the Chust Ghetto; conditions of the ghetto, such as the Jewish leadership; his assignment as a messenger and pass to leave the ghetto; smuggling in bread from outside the ghetto; people being punished for being Hungarian; being transported by train from the ghetto after three weeks; how partisans blew up the train and told prisoners to jump off and escape; arriving at Auschwitz in the spring of 1944; his memories of Dr.
- Interviewee
- Irving Schaffer
- Interviewer
- Mira Hodos
- Date
- interview: 1993 October 19
Physical Details
- Language
- English
- Extent
- 2 sound cassettes (90 min.).
Rights & Restrictions
- Conditions on Access
- There are no known restrictions on access to this material.
- Conditions on Use
- No restrictions on use
Keywords & Subjects
- Topical Term
- Antisemitism--Czechoslovakia.Escapes.Holocaust, Jewish (1939-1945)--Czechoslovakia--Personal narratives.Holocaust survivors--United States.Jewish councils--Ukraine--Khust.Jewish ghettos--Ukraine--Khust.Jews--Czechoslovakia.Jews--Education--Czechoslovakia.Jews--Ukraine--Khust.World War, 1939-1945--Concentration camps--Liberation.Men--Personal narratives.
- Geographic Name
- Khust (Ukraine)United States--Emigration and immigration.
- Personal Name
- Schaffer, Irving, 1928-
- Corporate Name
- American Jewish Joint Distribution CommitteeAuschwitz (Concentration camp)Dachau (Concentration camp)Mühldorf (Concentration camp)United Nations Relief and Rehabilitation Administration
A
List of Canadian Jews
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Il treno della salvezza
La questione ebraica
Tutto ciò che è necessario per il trionfo del male è che gli uomini buoni non facciano nulla.
Edmund Burke 1
La Palestina è la nostra casa storica sempre memorabile. Il nome stesso della Palestina sarebbe attirare la nostra gente con una forza di meravigliosa potenza. . . Dovremmo formare a parte di un baluardo dell'Europa contro l'Asia, un avamposto di civiltà in contrapposizione a barbarie. Come Stato neutrale dovremmo rimanere in contatto con tutta l'Europa, che dovrebbe garantire la nostra esistenza. . . Dovremmo formare una guardia d'onore su questi santuari, rispondendo dell'adempimento di questo dovere con la nostra esistenza. Questo guardia d'onore sarebbe il grande simbolo della soluzione della questione ebraica dopo diciotto secoli di sofferenza ebraica. Theodor Herzl, Lo Stato ebraico , 1896 Rezső Kasztner nasce nel 1906 a Kolozsvár, la città più grande della Transilvania, in Ungheria, e sita nei pressi del confine rumeno. I genitori ebrei che per gli attribuiscono tre nomi : Rudolf, per ricordare i Sassoni tedeschi; Rezső, per onorare i Magiari, e Israele, nome biblico di Giacobbe ricevuto quando ha combattuto e vinto l'Angelo delle Tenebre. Il padre Yitzhak, ricco mercante, trascorre le sue giornate nella sinagoga locale, studiando la Bibbia e discutendo con altri studiosi sull'interpretazione d'interi passi del Talmud. Gli affari vengono portati avanti dalla moglie, Helen, una donna istruita e con una buona predisposizione per gli affari che sogna di poter offrire ai suoi tre figli un'istruzione adeguata e un futuro migliore. Ben presto si rende conto che il primogenito, un ragazzo snello con i capelli lunghi, mossi e scuri, di bell'aspetto e una buona capacità di concentrazione. Oltre ad eccellere negli studi apprende e padroneggia ben cinque lingue: ungherese, rumeno, francese, tedesco e latino, per cui la madre gli suggerisce di intraprendere gli studi di