What happened to Hitler's family? Is progeria really accelerated aging?
What happened to Hitler's family? I know he had no siblings but surely there must have been others with the unfortunate surname. Were they persecuted or driven out of Germany/Austria?
What this tells you I don't know, but the rest of the world has shown a lot more interest in Adolf's relatives than Americans have. Historian Timothy Ryback brought the highbrows up to speed with a 2000 New Yorker article on the Hitler clan, but, U.S. tabloids having neglected the subject, ignorance remains high among the masses, including, if I may say so, you. No Hitler siblings? Nonsense--he had a sister, Paula, a half sister, Angela, and a half brother, Alois. Folks elsewhere are better informed. Over the past three years UK Third Reich buffs have had their choice of a book, a TV special, and various newspaper articles about the Hitler family's doings. But of course, you say--Europeans have reminders of World War II at every hand, history is more real to them, etc. Well, maybe. The fact remains that for years the descendants of some of Der Fuhrer's most obnoxious relatives have resided on Long Island, New York.
To be sure, obnoxiousness seems to be a Hitler family trait--Ryback says some of Adolf's relations still living in Austria hope to get their hands on royalties from Mein Kampf. (Lotsa luck--the cash and copyright were confiscated after the war.) The champs in the cheek department, however, were surely Brigid and William Patrick Hitler, the Nazi leader's sister-in-law and nephew, respectively. Brigid, born in Ireland, had eloped to London in 1910 with Alois Hitler, who sired William before bailing. In 1929 Willie began telling British reporters he was Hitler's nephew and allegedly tried to blackmail his uncle later. Adolf shut him up temporarily with money and jobs, but by 1939 Willie decided he'd do better working against the Nazis than for them and spent two years lecturing in the U.S. and Canada on the Hitler family's foibles. Brigid also attempted to cash in, first asking Hitler for money and when that went nowhere writing a book around 1940 claiming Hitler had visited her in pre-WWI Liverpool. Unpublished during her lifetime, the book came to historians' attention in the early 70s; some initially credited it, but I'd say most now regard it as a fraud.
After the war Hitler's relatives, including Brigid and Willie, decided to lay low, although in some cases not low enough--several in Europe died in Russian custody. Ryback tracked down Willie's sons on Long Island, but they wouldn't talk. (Brigid and Willie are long dead.) Journalist David Gardner has claimed that the sons agreed not to have children in order to end the Hitler bloodline. Still, it's premature to say we've heard the last of the house of Hitler. Ryback and another historian recently announced their discovery that Adolf had a mentally ill cousin. Far from profiting from her Hitler tie, in 1940 she was gassed.
Is progeria really what it looks like--that is, the accelerated aging of juveniles so that they die of old age in their childhood or teens? Or is it merely a matter of appearances? --Fayaway & Hermester Barrington, via e-mail
Kids with progeria are definitely aging--that's what makes them so interesting from a research standpoint. Due to a genetic defect, those with the most widely publicized form of the condition, Hutchinson-Gilford progeria syndrome, age roughly ten years physiologically for each year of life. You've probably seen pictures of kids with HGPS--bald and wrinkled, they look like wizened old men. It's not just appearances, either. Most people with HGPS die of heart attack or stroke, at an average age of 13.
In most cases what causes it all is a seemingly minor mutation in what's known as the LMNA gene, which generates a couple of proteins used to make the innermost layer (the lamina) of the membrane surrounding the body's cell nuclei (the nuclear envelope). The nuclear envelope regulates the flow of proteins into the nucleus, which is probably important to genetic expression as the cell reproduces. The mutation involves the substitution of a single nucleotide (thymine for cytosine, the T and C in the G-T-C-A alphabet blocks used to make all DNA) at one DNA location, codon 608. The change causes a bad coding splice, meaning that 50 amino acids get left out of a key protein in the lamina, with disastrous consequences for the cell's proprietor.
Extraordinary as it is that we've established all this, we're still not sure why lacking these amino acids leads to accelerated aging. Though HGPS is rare--only about 100 cases have been reported since it was first described in 1886, with fewer than 40 now living--it's one of the hot spots of genetic research, for the obvious reason that it could help us discover why we age and, conceivably, how to stop.